pubmed_id
stringlengths
39
43
abstract
stringlengths
3
18k
http://www.ncbi.nlm.nih.gov/pubmed/22480286
1. Cardiovasc Hematol Agents Med Chem. 2012 Jun;10(2):116-23. doi: 10.2174/187152512800388911. Dabigatran: a new chapter in anticoagulation. Ahmed S(1), Levin V, Malacoff R, Martinez MW. Author information: (1)Division of Cardiology, Lehigh Valley Health Network, Allentown, PA 18103, USA. For the last 60 years warfarin has been the cornerstone for chronic anticoagulation in prevention of ischemic strokes and systemic embolization. Warfarin therapy has several limitations including frequent monitoring and various food and significant drug interactions, which make it a less than ideal chronic oral anticoagulant. The continued search for safe, effective, medications with predictable pharmacokinetic profiles has led to newer alternatives. Dabigatran is a potent reversible, competitive direct thrombin inhibitor which is available as the prodrug, Dabigatran etexilate. It was first approved in Europe and recently in October 2010, the US food and drug administration (FDA) has approved the use of this novel oral anticoagulation for prevention of stroke in those with non valvular atrial fibrillation. This review will cover the chemical structure, mechanism of action, pharmacokinetic profile, clinical trials, dosage, clinical implication and adverse effects of dabigatran. DOI: 10.2174/187152512800388911 PMID: 22480286 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/23328389
1. J Cardiovasc Pharmacol. 2013 Jun;61(6):474-81. doi: 10.1097/FJC.0b013e31828685da. Pharmacokinetics of oral treprostinil sustained release tablets during chronic administration to patients with pulmonary arterial hypertension. White RJ(1), Torres F, Allen R, Jerjes C, Pulido T, Yehle D, Howell M, Laliberte K, Marier JF, Tapson VF. Author information: (1)Division of Pulmonary & Critical Care Medicine, University of Rochester, Rochester, NY 14623, USA. jim_white@urmc.rochester.edu Comment in J Cardiovasc Pharmacol. 2013 Jun;61(6):471-3. doi: 10.1097/FJC.0b013e318294980e. Pulmonary arterial hypertension (PAH) is a progressive vascular disease that ultimately leads to right ventricular failure and death. Treprostinil diolamine is an oral prostacyclin analogue; sustained release tablets of oral treprostinil are currently being evaluated for efficacy and safety as a potential therapy in patients with PAH. Previous attempts at developing an oral prostanoid have been limited by rapid absorption and short plasma half-life; thus, the aim of this study was to characterize the pharmacokinetic profile of treprostinil diolamine in PAH patients after chronic dosing. The study enrolled 74 PAH patients who had been taking treprostinil diolamine for a minimum of 4 weeks (range: 0.5-16 mg). We collected plasma samples over 12 hours and estimated pharmacokinetic parameters using noncompartmental methods. Seventy patients had complete data. After chronic twice-daily oral dosing of treprostinil diolamine, mean area under the curve (AUC0-12) of treprostinil increased from 5244 to 20,4086 pg·hr-·mL- and mean maximum observed plasma concentration (Cmax) increased from 1383 to 33588 pg/mL. The apparent clearance (CL/F) was similar across all doses, indicating a linear dose-exposure relationship after twice-daily dosing. We conclude that twice-daily oral treprostinil provides sustained and proportional treprostinil concentrations over a wide range of doses during chronic administration to PAH patients. DOI: 10.1097/FJC.0b013e31828685da PMID: 23328389 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/15831522
1. Mol Endocrinol. 2005 Jun;19(6):1618-28. doi: 10.1210/me.2004-0503. Epub 2005 Apr 14. Thyroid hormone induces cardiac myocyte hypertrophy in a thyroid hormone receptor alpha1-specific manner that requires TAK1 and p38 mitogen-activated protein kinase. Kinugawa K(1), Jeong MY, Bristow MR, Long CS. Author information: (1)Denver Health Medical Center, Colorado 80204, USA. Alterations in TR [thyroid hormone (TH) receptor]1 isoform expression have been reported in models of both physiologic and pathologic cardiac hypertrophy as well as in patients with heart failure. In this report, we demonstrate that TH induces hypertrophy as a direct result of binding to the TRalpha1 isoform and, moreover, that overexpression of TRalpha1 alone is also associated with a hypertrophic phenotype, even in the absence of ligand. The mechanism of TH and TRalpha1-specific hypertrophy is novel for a nuclear hormone receptor and involves the transforming growth factor beta-activated kinase (TAK1) and p38. Mitigating TRalpha1 effects, both TRalpha2 and TRbeta1 attenuate TRalpha1-induced myocardial growth and gene expression by diminishing TAK1 and p38 activities, respectively. These findings refine our previous observations on TR expression in the hypertrophied and failing heart and suggest that manipulation of thyroid hormone signaling in an isoform-specific manner may be a relevant therapeutic target for altering the pathologic myocardial program. DOI: 10.1210/me.2004-0503 PMCID: PMC1237131 PMID: 15831522 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/21988948
1. Crit Pathw Cardiol. 2011 Jun;10(2):84-6. doi: 10.1097/HPC.0b013e318224df28. Dabigatran: a new option for anticoagulation in atrial fibrillation and venous thromboembolism. Acharjee S(1), Cannon CP. Author information: (1)Department of Medicine, SUNY at Buffalo School of Medicine, NY, USA. For several decades now, oral anticoagulation with warfarin has represented the cornerstone of measures to prevent occurrence of ischemic stroke in high-risk patients with atrial fibrillation. However, the mechanism of action and pharmacokinetic profile of this vitamin K antagonist confers a narrow therapeutic range and makes it prone to drug and dietary interactions, requiring frequent monitoring of its effectiveness. The recently introduced oral direct thrombin antagonist, dabigatran, has been shown in phase III clinical trials to be noninferior in efficacy to warfarin for the prevention of thromboembolic events in patients with atrial fibrillation, as well as in treatment of acute venous thromboembolism. In this article, we review the factors necessitating the development of dabigatran, summarize key clinical trial evidence leading to its approval, and discuss its potential role as an alternative to warfarin in current clinical practice. DOI: 10.1097/HPC.0b013e318224df28 PMID: 21988948 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/17103451
1. Am J Med Genet A. 2006 Dec 15;140(24):2730-41. doi: 10.1002/ajmg.a.31530. Clinical features and management issues in Mowat-Wilson syndrome. Adam MP(1), Schelley S, Gallagher R, Brady AN, Barr K, Blumberg B, Shieh JT, Graham J, Slavotinek A, Martin M, Keppler-Noreuil K, Storm AL, Hudgins L. Author information: (1)Department of Human Genetics, Emory University School of Medicine, Atlanta, Georgia, USA. madam@genetics.emory.edu Comment in Am J Med Genet A. 2007 Jul 1;143A(13):1528-30. doi: 10.1002/ajmg.a.31801. Mowat-Wilson syndrome (MWS) is a relatively newly described multiple congenital anomaly/mental retardation syndrome. Haploinsufficiency of a gene termed ZFHX1B (also known as SIP1) on chromosome 2 is responsible for this condition, and clinical genetic testing for MWS recently became available. The majority of reports in the literature originate from Northern Europe and Australia. Here we report our clinical experience with 12 patients diagnosed with MWS within a 2-year period of time in the United States, with particular emphasis on clinical characteristics and management strategies. Individuals with this condition have characteristic facial features, including microcephaly, hypertelorism, medially flared and broad eyebrows, prominent columella, pointed chin, and uplifted earlobes, which typically prompt the clinician to consider the diagnosis. Medical issues in our cohort of patients included seizures (75%) with no predeliction for any particular seizure type; agenesis of the corpus callosum (60% of our patients studied); congenital heart defects (75%), particularly involving the pulmonary arteries and/or valves; hypospadias (55% of males); severely impaired or absent speech (100% of individuals over 1 year of age) with relatively spared receptive language; and Hirschsprung disease (50%) or chronic constipation (25%). The incidence of MWS is unknown, but based on the number of patients identified in a short period of time within the US, it is likely greatly under recognized. MWS should be considered in any individual with severely impaired or absent speech, especially in the presence of seizures and anomalies involving the pulmonary arteries (particularly pulmonary artery sling) or pulmonary valves. (c) 2006 Wiley-Liss, Inc. DOI: 10.1002/ajmg.a.31530 PMID: 17103451 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/3931221
1. Science. 1985 Nov 1;230(4725):550-3. doi: 10.1126/science.3931221. Cyanobacterial light-harvesting complex subunits encoded in two red light-induced transcripts. Conley PB, Lemaux PG, Grossman AR. The major light-harvesting complex in cyanobacteria and red algae, the phycobilisome, is composed of chromophoric and nonchromophoric polypeptides. Two linked genes encoding major chromophoric components, the polypeptide subunits of phycocyanin, were isolated from the cyanobacterium Fremyella diplosiphon. Transcripts from this phycocyanin subunit gene cluster were present as major species in the cyanobacterium grown in red light, but not in cultures maintained in green light. The genes for the subunits of the red light-induced phycocyanin were transcribed together (beta-phycocyanin followed by alpha-phycocyanin) on two messenger RNA species; one contained 1600 bases while the other had 3800 bases. The latter, which encompassed the smaller transcript, contained additional sequences extending from the 3' end of the coding region of the alpha-phycocyanin gene. It may encode other light-induced components of the phycobilisome. Since phycocyanin, which effectively absorbs red light, becomes a dominant constituent of the phycobilisome in red light, these different levels may reflect an important adaptive mechanism of these organisms to their environment. DOI: 10.1126/science.3931221 PMID: 3931221 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/7678762
1. Plant Mol Biol. 1993 Jan;21(1):27-38. doi: 10.1007/BF00039615. Characterization and transcript analysis of the major phycobiliprotein subunit genes from Aglaothamnion neglectum (Rhodophyta). Apt KE(1), Grossman AR. Author information: (1)Carnegie Institution of Washington, Department of Plant Biology, Stanford, CA 94305-1297. The genes encoding the alpha and beta subunits of allophycocyanin, phycocyanin and phycoerythrin from the red alga Aglaothamnion neglectum were isolated and characterized. While the operons containing the different phycobiliprotein genes are dispersed on the plastid genome, the genes encoding the alpha and beta subunits for each phycobiliprotein are contiguous. The beta subunit gene is 5' for both the phycocyanin and phycoerythrin operons, while the alpha subunit gene is 5' for the allophycocyanin operon. The amino acid sequences of A. neglectum phycobiliproteins, as deduced from the nucleotide sequences of the genes, are 65-85% identical to analogous proteins from other red algae and cyanobacteria. The conserved nature of the plastid-encoded red algal and cyanobacterial phycobiliprotein genes supports the proposed origin of red algal plastids from cyanobacterial endosymbionts. Many environmental factors effect phycobilisome biosynthesis. The effect of both nutrient availability and light quantity on the level of A. neglectum phycobiliprotein subunits and the mRNA species encoding those subunits is described. DOI: 10.1007/BF00039615 PMID: 7678762 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/20074983
1. Blood Cells Mol Dis. 2010 Mar 15;44(3):181-7. doi: 10.1016/j.bcmd.2009.12.006. Epub 2010 Jan 13. Spontaneous regression of disease manifestations can occur in type 1 Gaucher disease; results of a retrospective cohort study. Boomsma JM(1), van Dussen L, Wiersma MG, Groener JE, Aerts JM, Maas M, Hollak CE. Author information: (1)Department of Internal Medicine, Academic Medical Center, Amsterdam, The Netherlands. Gaucher disease (GD) is a lysosomal storage disorder, caused by deficient activity of the enzyme glucocerebrosidase. GD is classically divided into three major phenotypes. The most prevailing form is type 1, which presents with variable hepatosplenomegaly, cytopenia, and/or bone disease. In adult patients with mild manifestations, progress of disease might be slow or even absent. As a consequence, treatment with intravenous enzyme replacement or substrate reduction is not always necessary. In the Netherlands, the follow-up of GD patients is centralized, which allows detailed investigation of untreated patients. A retrospective study was conducted in 18 type 1 GD patients, (2 teenagers: 15 and 16 years of age at first visit) who were not treated for at least one year. The chitotriosidase activity, platelet count, hemoglobin level, lumbar bone marrow fat content measured with quantitative chemical shift imaging (QCSI), liver ratio (ml/kg body weight), and spleen volume were recorded. Criteria were developed to score regression, stability or progression of disease. During a mean follow up of 4.5 years (range 1.1-12.2) seven patients (39%) showed spontaneous regression of GD. Eight patients (44%) were stable. Two patients had progressive disease, solely based upon a sustained increase in chitotriosidase activity. A pediatric patient had an increase in splenomegaly but an improvement in bone marrow fat fraction, probably due to aging. Nine patients fulfilled the local criteria to start treatment at first visit, of whom six started treatment within 1.1 to 6.8 years. The other three refused therapy, but nevertheless showed stability or even regression of the disease during a follow up of 4.6, 9.5 and 11.4 years respectively. None of the parameters was predictive of progression or regression of disease. In conclusion, GD in adults can, in some cases, regress spontaneously. No parameters for accurately predicting future disease course exist. 2009 Elsevier Inc. All rights reserved. DOI: 10.1016/j.bcmd.2009.12.006 PMID: 20074983 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/21526168
1. PLoS One. 2011 Apr 22;6(4):e17626. doi: 10.1371/journal.pone.0017626. Evaluation of the efficacy and safety of rivaroxaban using a computer model for blood coagulation. Burghaus R(1), Coboeken K, Gaub T, Kuepfer L, Sensse A, Siegmund HU, Weiss W, Mueck W, Lippert J. Author information: (1)Bayer Schering Pharma AG, Wuppertal, Germany. Rivaroxaban is an oral, direct Factor Xa inhibitor approved in the European Union and several other countries for the prevention of venous thromboembolism in adult patients undergoing elective hip or knee replacement surgery and is in advanced clinical development for the treatment of thromboembolic disorders. Its mechanism of action is antithrombin independent and differs from that of other anticoagulants, such as warfarin (a vitamin K antagonist), enoxaparin (an indirect thrombin/Factor Xa inhibitor) and dabigatran (a direct thrombin inhibitor). A blood coagulation computer model has been developed, based on several published models and preclinical and clinical data. Unlike previous models, the current model takes into account both the intrinsic and extrinsic pathways of the coagulation cascade, and possesses some unique features, including a blood flow component and a portfolio of drug action mechanisms. This study aimed to use the model to compare the mechanism of action of rivaroxaban with that of warfarin, and to evaluate the efficacy and safety of different rivaroxaban doses with other anticoagulants included in the model. Rather than reproducing known standard clinical measurements, such as the prothrombin time and activated partial thromboplastin time clotting tests, the anticoagulant benchmarking was based on a simulation of physiologically plausible clotting scenarios. Compared with warfarin, rivaroxaban showed a favourable sensitivity for tissue factor concentration inducing clotting, and a steep concentration-effect relationship, rapidly flattening towards higher inhibitor concentrations, both suggesting a broad therapeutic window. The predicted dosing window is highly accordant with the final dose recommendation based upon extensive clinical studies. DOI: 10.1371/journal.pone.0017626 PMCID: PMC3081290 PMID: 21526168 [Indexed for MEDLINE] Conflict of interest statement: Competing Interests: All authors are Bayer AG subsidiary employees and own stocks of Bayer AG. This does not alter the authors’ adherence to all the PLoS ONE policies on sharing data and materials.
http://www.ncbi.nlm.nih.gov/pubmed/2502578
1. J Immunol Methods. 1989 Jul 6;121(1):9-18. doi: 10.1016/0022-1759(89)90414-6. A novel and inexpensive source of allophycocyanin for multicolor flow cytometry. Jung TM(1), Dailey MO. Author information: (1)Department of Pathology, University of Iowa, College of Medicine, Iowa City 52242. Allophycocyanin (APC) belongs to a family of phycobiliproteins that are well suited as fluorescent reagents for flow cytometric analysis, since they have a broad excitation spectrum, a large Stoke's shift and they fluoresce with a high quantum yield. The widespread use of APC has been limited by the availability of raw material and high cost of the purified phycobiliprotein. We have assessed the suitability of dry, powdered Spirulina platensis, available at health food stores, as an inexpensive source of APC. APC was extracted from Spirulina platensis by overnight treatment with lysozyme, followed by ammonium sulfate precipitation. APC was then separated from phycocyanin (the only other major phycobiliprotein in Spirulina) by elution of bound material from an hydroxylapatite column using an increasing continuous phosphate gradient. APC isolated in this manner retained its normal trimeric structure. The absorbance and fluorescence excitation and emission spectra of the purified phycobiliproteins were identical to those previously shown for C-PC and APC. APC can be stored concentrated at 4 degrees C, frozen at -70 degrees C, or as a saturated ammonium sulfate precipitate, with no subunit dissociation or change in spectral properties. Moreover, APC has been conjugated to monoclonal and polyclonal antibodies for use in multicolor FACS analysis, with the conjugated antibody activity remaining stable for at least 2 years. Thus, this procedure is a simple, cost-effective method for preparing reagents for multicolor immunofluorescence and flow cytometry. DOI: 10.1016/0022-1759(89)90414-6 PMID: 2502578 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/17010801
1. J Am Coll Cardiol. 2006 Oct 3;48(7):1396-8. doi: 10.1016/j.jacc.2006.07.016. Epub 2006 Sep 12. Phospholamban R14 deletion results in late-onset, mild, hereditary dilated cardiomyopathy. DeWitt MM(1), MacLeod HM, Soliven B, McNally EM. Author information: (1)Department to Medicine, Section of Cardiology, The University of Chicago, Chicago, Illinois, USA. OBJECTIVES: The purpose of this research was to determine the phenotypic spectrum associated with phospholamban gene (PLN) mutations. BACKGROUND: Inheritance contributes to the development of dilated cardiomyopathy. Mutations in the gene encoding PLN have been associated with dilated cardiomyopathy characterized by early onset and the presence of lethal ventricular arrhythmias. METHODS: We screened a cohort of 260 unrelated dilated cardiomyopathy patients from a tertiary care referral center for mutations in the PLN gene. RESULTS: Family history of cardiomyopathy was present in approximately one-half the individuals in this cohort. We identified 1 family with a deletion of arginine 14 in the PLN. Interestingly, unlike other individuals reported with the identical PLN mutation, these individuals were not diagnosed with dilated cardiomyopathy until their seventh decade when they were only mildly symptomatic with congestive heart failure. CONCLUSIONS: The identical PLN mutation can be associated with both mild and severe forms of dilated cardiomyopathy. Additionally, PLN mutations should be considered in late onset cardiomyopathy. (Genetics of Cardiovascular and Neuromuscular Disease; http://www.clinicaltrials.gov/ct/show/NCT00138931?order=1; NCT00138931) DOI: 10.1016/j.jacc.2006.07.016 PMID: 17010801 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/18989393
1. PLoS Comput Biol. 2008 Nov;4(11):e1000213. doi: 10.1371/journal.pcbi.1000213. Epub 2008 Nov 7. Transmembrane topology and signal peptide prediction using dynamic bayesian networks. Reynolds SM(1), Käll L, Riffle ME, Bilmes JA, Noble WS. Author information: (1)Department of Electrical Engineering, University of Washington, Seattle, Washington, United States of America. Hidden Markov models (HMMs) have been successfully applied to the tasks of transmembrane protein topology prediction and signal peptide prediction. In this paper we expand upon this work by making use of the more powerful class of dynamic Bayesian networks (DBNs). Our model, Philius, is inspired by a previously published HMM, Phobius, and combines a signal peptide submodel with a transmembrane submodel. We introduce a two-stage DBN decoder that combines the power of posterior decoding with the grammar constraints of Viterbi-style decoding. Philius also provides protein type, segment, and topology confidence metrics to aid in the interpretation of the predictions. We report a relative improvement of 13% over Phobius in full-topology prediction accuracy on transmembrane proteins, and a sensitivity and specificity of 0.96 in detecting signal peptides. We also show that our confidence metrics correlate well with the observed precision. In addition, we have made predictions on all 6.3 million proteins in the Yeast Resource Center (YRC) database. This large-scale study provides an overall picture of the relative numbers of proteins that include a signal-peptide and/or one or more transmembrane segments as well as a valuable resource for the scientific community. All DBNs are implemented using the Graphical Models Toolkit. Source code for the models described here is available at http://noble.gs.washington.edu/proj/philius. A Philius Web server is available at http://www.yeastrc.org/philius, and the predictions on the YRC database are available at http://www.yeastrc.org/pdr. DOI: 10.1371/journal.pcbi.1000213 PMCID: PMC2570248 PMID: 18989393 [Indexed for MEDLINE] Conflict of interest statement: The authors have declared that no competing interests exist.
http://www.ncbi.nlm.nih.gov/pubmed/21297619
1. Nat Methods. 2011 Mar;8(3):260-6. doi: 10.1038/nmeth.1567. Epub 2011 Feb 6. Flybow: genetic multicolor cell labeling for neural circuit analysis in Drosophila melanogaster. Hadjieconomou D(1), Rotkopf S, Alexandre C, Bell DM, Dickson BJ, Salecker I. Author information: (1)Medical Research Council National Institute for Medical Research, Division of Molecular Neurobiology, London, UK. Comment in Nat Methods. 2011 Mar;8(3):217-8. doi: 10.1038/nmeth0311-217. To facilitate studies of neural network architecture and formation, we generated three Drosophila melanogaster variants of the mouse Brainbow-2 system, called Flybow. Sequences encoding different membrane-tethered fluorescent proteins were arranged in pairs within cassettes flanked by recombination sites. Flybow combines the Gal4-upstream activating sequence binary system to regulate transgene expression and an inducible modified Flp-FRT system to drive inversions and excisions of cassettes. This provides spatial and temporal control over the stochastic expression of one of two or four reporters within one sample. Using the visual system, the embryonic nervous system and the wing imaginal disc, we show that Flybow in conjunction with specific Gal4 drivers can be used to visualize cell morphology with high resolution. Finally, we demonstrate that this labeling approach is compatible with available Flp-FRT-based techniques, such as mosaic analysis with a repressible cell marker; this could further support the genetic analysis of neural circuit assembly and function. DOI: 10.1038/nmeth.1567 PMID: 21297619 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/9548282
1. Electrophoresis. 1998 Feb;19(2):215-9. doi: 10.1002/elps.1150190213. Electrophoretic applications of phycobiliproteins. Aráoz R(1), Lebert M, Häder DP. Author information: (1)Institut für Botanik und Pharmazeutische Biologie, Friedrich-Alexander-Universität, Erlangen, Germany. Phycobiliproteins are homologous chromoproteins which constitute the phycobilisomes, the light harvesting complexes of the photosynthetic apparatus in cyanobacteria, rhodophyta and cryptophyta. In the present work, phycocyanin (PC) and phycoerythrin (PE) from a Nostoc species are proposed as protein markers for electrophoretic techniques. Phycocyanin is a blue-colored phycobiliprotein; it carries phycocyanobilin as chromophoric group and is composed of two subunits, alpha and beta, with Mr of 14000 and 17000, respectively. In contrast, the PE subunits, having a similar Mr of 21000, are deep rose chromoproteins and carry phycoerythrobilin residues. Both low molecular weight phycobiliproteins are also suitable for monitoring protein blotting and the focusing time of protein samples during isoelectric focusing as internal markers. The PE subunits which form a single broad band after sodium dodecyl sulfate-polyacrylamide gel electrophoresis have different isoelectric points, and they form two visible bands when they reach their isoelectric point. The phycobilisomes constitute up to 50% of the total protein in cyanobacteria and their content in PC or PE can be up- or down-regulated by using different light conditions (chromatic adaptation). DOI: 10.1002/elps.1150190213 PMID: 9548282 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/15302727
1. Chest. 2004 Aug;126(2):420-7. doi: 10.1378/chest.126.2.420. Treprostinil, a prostacyclin analogue, in pulmonary arterial hypertension associated with connective tissue disease. Oudiz RJ(1), Schilz RJ, Barst RJ, Galié N, Rich S, Rubin LJ, Simonneau G; Treprostinil Study Group. Author information: (1)Research and Education Institute, Harbor-UCLA Medical Center, Torrance, CA 90502, USA. oudiz@humc.edu Comment in Chest. 2005 Sep;128(3):1888. doi: 10.1378/chest.128.3.1888. STUDY OBJECTIVES: To assess the efficacy and safety of continuous subcutaneous infusion of treprostinil, a stable prostacyclin analogue, for treating pulmonary arterial hypertension (PAH) in patients with connective tissue disease (CTD). DESIGN: Two multicenter, randomized, double-blind, placebo-controlled, prospective trials of treprostinil vs placebo in 470 patients with PAH. PATIENTS: A subset of 90 patients with PAH and CTD, including systemic lupus erythematosus, diffuse scleroderma, limited scleroderma, and mixed CTD/overlap syndrome. INTERVENTIONS: Patients received either treprostinil (initiated at 1.25 ng/kg/min, and titrated upward) or placebo via continuous subcutaneous infusion. The maximum dose of treprostinil allowed was 22.5 ng/kg/min. MEASUREMENTS: Six-minute walk (6MW) distance and dyspnea-fatigue scores were determined at baseline, and at 6 weeks and 12 weeks. Hemodynamic measures were obtained at baseline and at 12 weeks. RESULTS: At baseline, most patients had New York Heart Association class III symptoms. The mean baseline 6MW distance was 289 m (range, 60 to 448 m). The mean dose of treprostinil at week 12 was 8.4 ng/kg/min (range, 1.25 to 17.5 ng/kg/min). After 12 weeks, the change in cardiac index from baseline was + 0.2 +/- 0.08 L/min/m(2) in the treprostinil group and - 0.07 +/- 0.07 L/min/m(2) in the placebo group (p = 0.007). The pulmonary vascular resistance index decreased by 4 +/- 2 U x m(2) in the treprostinil group and increased by 1 +/- 1 U x m(2) in the placebo group (p = 0.006). The placebo-corrected median improvement from baseline in 6MW distance was 25 m in treprostinil-treated patients (p = 0.055); this improvement appeared to be dose related. Dyspnea fatigue scores also improved in the treprostinil group compared with the placebo group (p = 0.014). Adverse events included infusion site pain and typical side effects related to prostaglandins, and were tolerated by most patients. CONCLUSIONS: Continuous subcutaneous infusion of treprostinil in patients with PAH associated with CTD improved exercise capacity, symptoms of PAH, and hemodynamics. DOI: 10.1378/chest.126.2.420 PMID: 15302727 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/1859661
1. Eur J Cardiothorac Surg. 1991;5(5):230-4. doi: 10.1016/1010-7940(91)90169-k. Coronary artery bypass surgery in patients with angina pectoris and hypothyroidism. Kawasuji M(1), Sawa S, Tsujiguchi H, Iwa T. Author information: (1)Department of Surgery I, Kanazawa University School of Medicine, Japan. The treatment of hypothyroidism in patients undergoing coronary artery bypass surgery is a difficult clinical problem. To determine perioperative thyroid replacement therapy in patients with hypothyroidism, plasma total thyroxine (T4), total triiodothyroxine (T3), free T4, free T3 and thyroid-stimulating hormone levels were measured preoperatively and at 1, 2, 3, 7, and 14 days after operation in 9 patients with hypothyroidism and were compared with levels in 14 patients with normal thyroid function who underwent coronary bypass surgery. In the normal control group, total T4 decreased to its lowest level on the 1st postoperative day and then increased gradually to the preoperative level at 7 days. Total T4 remained within the normal range throughout the entire postoperative course. In 6 patients with hypothyroidism who were treated with thyroid hormone before surgery, total T4 decreased immediately after operation and only increased after starting thyroid replacement therapy. In 3 hypothyroid patients without prior thyroid replacement, total T4 showed a change similar to patients in the control group but remained below the normal range until starting thyroid replacement therapy. Coronary bypass surgery was performed safely in patients with hypothyroidism. Preoperative thyroid replacement with suboptimal doses was safe in patients with severe hypothyroidism. Adequate postoperative thyroid replacement was achieved in all patients without complications. DOI: 10.1016/1010-7940(91)90169-k PMID: 1859661 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/23827424
1. Pediatr Neurol. 2013 Jul;49(1):31-39.e2. doi: 10.1016/j.pediatrneurol.2013.02.015. Cerebrospinal fluid brain injury biomarkers in children: a multicenter study. Shahim P(1), Darin N, Andreasson U, Blennow K, Jennions E, Lundgren J, Månsson JE, Naess K, Törnhage CJ, Zetterberg H, Mattsson N. Author information: (1)Clinical Neurochemistry Laboratory, Institute of Neuroscience and Physiology, Department of Neurochemistry, Sahlgrenska University Hospital/Mölndal, Sweden. pashtun.shahim@neuro.gu.se BACKGROUND: Cerebrospinal fluid (CSF) biomarkers reflecting neuronal and astroglial injury, such as total tau (T-tau), glial fibrillary acidic protein (GFAP), and neurofilament light (NFL), have been extensively investigated in neurologic diseases in adults, but no large study has investigated these biomarkers in children. METHODS: This study presents a detailed evaluation of CFS T-tau, GFAP, NFL, and CSF:albumin ratio in a large cohort of pediatric patients. This is a retrospective multicenter study on pediatric patients aged <16 years (n = 607), where neuronal injury biomarkers T-tau, GFAP, NFL, and CSF albumin ratio were analyzed during 2000-2010 at the Clinical Neurochemistry Laboratory, Sahlgrenska University Hospital, Sweden. The patients were grouped into eight categories: epilepsy, infectious and inflammatory central nervous system disorders, progressive encephalopathy, static encephalopathy, tumors, movement disorders, miscellaneous disorders, and a control group. RESULTS: T-tau, GFAP, and NFL were increased in progressive encephalopathy (P < 0.001), epilepsy (P < 0.001), and infectious and inflammatory central nervous system disorders (P < 0.001) compared with controls. T-tau was the biomarker with the highest diagnostic accuracy with the area under the curve of 0.83 (95% confidence interval (CI), 0.77-0.90; P < 0.0001) for progressive encephalopathy followed by epilepsy 0.80 (95% CI, 0.75-0.87; P < 0.0001). The combination of all four biomarkers further improved the area under the curve for the progressive encephalopathy 0.87 (95% CI, 0.77-0.89; P < 0.0001), followed by epilepsy 0.81 (95% CI, 0.74-0.80; P = 0.030). The combination of the biomarkers also separated progressive from static encephalopathy 0.88 (95% CI, 0.83-0.93; P < 0.0001). CONCLUSIONS: CSF T-tau, GFAP, and NFL are differently altered across different neurologic diseases in children. Importantly, the biomarker pattern distinguishes between progressive and static neurologic disorders. Copyright © 2013 Elsevier Inc. All rights reserved. DOI: 10.1016/j.pediatrneurol.2013.02.015 PMID: 23827424 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/10339434
1. Curr Biol. 1999 May 20;9(10):531-4. doi: 10.1016/s0960-9822(99)80237-1. Thermostable uracil-DNA glycosylase from Thermotoga maritima a member of a novel class of DNA repair enzymes. Sandigursky M(1), Franklin WA. Author information: (1)Departments of Radiology and Radiation Oncology, Albert Einstein College of Medicine, 1300 Morris Park Avenue, Bronx, New York 10461, USA. Uracil-DNA glycosylase (UDG) is a ubiquitous enzyme found in eukaryotes and prokaryotes [1][2][3]. This enzyme removes uracil bases that are present in DNA as a result of either deamination of cytosine or misincorporation of dUMP instead of dTMP [4] [5], and it is the primary activity in the DNA base excision repair pathway. Although UDG activities have been shown to be present in several thermophiles [6][7][8], no sequences have been found that are complementary to the Escherichia coli ung gene, which encodes UDG [9]. Here, we describe a UDG from the thermophile Thermotoga maritima. The T. maritima UDG gene has a low level of homology to the E. coli G-T/U mismatch-specific DNA glycosylase gene (mug). The expressed protein is capable of removing uracil from DNA containing either a U-A or a U-G base pair and is heat-stable up to 75 degrees C. The enzyme is also active on single-stranded DNA containing uracil. Analogous genes appear to be present in several prokaryotic organisms, including thermophilic and mesophilic eubacteria as well as archaebacteria, the human-disease pathogens Treponema palladium and Rickettsia prowazekii, and the extremely radioresistant organism Deinococcus radiodurans. These findings suggest that the T. maritima UDG is a member of a new class of DNA repair enzymes. DOI: 10.1016/s0960-9822(99)80237-1 PMID: 10339434 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/26052092
1. Cancer Lett. 2015 Sep 1;365(2):141-8. doi: 10.1016/j.canlet.2015.06.003. Epub 2015 Jun 5. Circular RNA: A new star of noncoding RNAs. Qu S(1), Yang X(1), Li X(1), Wang J(1), Gao Y(1), Shang R(1), Sun W(1), Dou K(1), Li H(2). Author information: (1)Department of Hepatobiliary Surgery, Xijing Hospital, The Fourth Military Medical University, Xi'an, 710032, China. (2)Department of Hepatobiliary Surgery, Xijing Hospital, The Fourth Military Medical University, Xi'an, 710032, China. Electronic address: lihaim@fmmu.edu.cn. Comment in BJOG. 2016 Dec;123(13):2119. doi: 10.1111/1471-0528.13965. Circular RNAs (circRNAs) are a novel type of RNA that, unlike linear RNAs, form a covalently closed continuous loop and are highly represented in the eukaryotic transcriptome. Recent studies have discovered thousands of endogenous circRNAs in mammalian cells. CircRNAs are largely generated from exonic or intronic sequences, and reverse complementary sequences or RNA-binding proteins (RBPs) are necessary for circRNA biogenesis. The majority of circRNAs are conserved across species, are stable and resistant to RNase R, and often exhibit tissue/developmental-stage-specific expression. Recent research has revealed that circRNAs can function as microRNA (miRNA) sponges, regulators of splicing and transcription, and modifiers of parental gene expression. Emerging evidence indicates that circRNAs might play important roles in atherosclerotic vascular disease risk, neurological disorders, prion diseases and cancer; exhibit aberrant expression in colorectal cancer (CRC) and pancreatic ductal adenocarcinoma (PDAC); and serve as diagnostic or predictive biomarkers of some diseases. Similar to miRNAs and long noncoding RNAs (lncRNAs), circRNAs are becoming a new research hotspot in the field of RNA and could be widely involved in the processes of life. Herein, we review the formation and properties of circRNAs, their functions, and their potential significance in disease. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved. DOI: 10.1016/j.canlet.2015.06.003 PMID: 26052092 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/24523921
1. PLoS One. 2014 Feb 11;9(2):e88591. doi: 10.1371/journal.pone.0088591. eCollection 2014. Biomarker evidence of axonal injury in neuroasymptomatic HIV-1 patients. Jessen Krut J(1), Mellberg T(1), Price RW(2), Hagberg L(1), Fuchs D(3), Rosengren L(4), Nilsson S(5), Zetterberg H(6), Gisslén M(1). Author information: (1)Institute of Biomedicine, Department of Infectious Diseases, University of Gothenburg, Gothenburg, Sweden. (2)Department of Neurology, University of California San Francisco, San Francisco, California, United States of America. (3)Division of Biological Chemistry, Biocenter, Innsbruck Medical University, Innsbruck, Austria. (4)Institute of Neuroscience and Physiology, Department of Neurology, the Sahlgrenska Academy at the University of Gothenburg, Gothenburg, Sweden. (5)Mathematical Sciences, Chalmers University of Technology, Gothenburg, Sweden. (6)Institute of Neuroscience and Physiology, Department of Psychiatry and Neurochemistry, the Sahlgrenska Academy at the University of Gothenburg, Gothenburg, Sweden ; UCL Institute of Neurology, Queen Square, London, United Kingdom. BACKGROUND: Prevalence of neurocognitive impairment in HIV-1 infected patients is reported to be high. Whether this is a result of active HIV-related neurodegeneration is unclear. We examined axonal injury in HIV-1 patients by measuring the light subunit of neurofilament protein (NFL) in CSF with a novel, sensitive method. METHODS: With a cross-sectional design, CSF concentrations of neurofilament protein light (NFL) (marker of neuronal injury), neopterin (intrathecal immunoactivation) and CSF/Plasma albumin ratio (blood-brain barrier integrity) were analyzed on CSF from 252 HIV-infected patients, subdivided into untreated neuroasymptomatics (n = 200), HIV-associated dementia (HAD) (n = 14) and on combinations antiretroviral treatment (cART) (n = 85), and healthy controls (n = 204). 46 HIV-infected patients were included in both treated and untreated groups, but sampled at different timepoints. Furthermore, 78 neuroasymptomatic patients were analyzed before and after treatment initiation. RESULTS: While HAD patients had the highest NFL concentrations, elevated CSF NFL was also found in 33% of untreated neuroasymptomatic patients, mainly in those with blood CD4+ cell counts below 250 cells/μL. CSF NFL concentrations in the untreated neuroasymptomatics and treated groups were equivalent to controls 18.5 and 3.9 years older, respectively. Neopterin correlated with NFL levels in untreated groups while the albumin ratio correlated with NFL in both untreated and treated groups. CONCLUSIONS: Increased CSF NFL indicates ongoing axonal injury in many neuroasymptomatic patients. Treatment decreases NFL, but treated patients retain higher levels than controls, indicating either continued virus-related injury or an aging-like effect of HIV infection. NFL correlates with neopterin and albumin ratio, suggesting an association between axonal injury, neuroinflammation and blood-brain barrier permeability. NFL appears to be a sensitive biomarker of subclinical and clinical brain injury in HIV and warrants further assessment for broader clinical use. DOI: 10.1371/journal.pone.0088591 PMCID: PMC3921217 PMID: 24523921 [Indexed for MEDLINE] Conflict of interest statement: Competing Interests: Dr Jan Krut, Dr Tomas Mellberg, Dr Dietmar Fuchs, Dr Lars Rosengren, Dr Staffan Nilsson and Dr Henrik Zetterberg have all declared that no competing interests exist. Dr Richard W Price has the following conflicts: Serving as a Consultant to Merck & Co and receiving honoraria and travel reimbursement for meeting presentations from AbbVie. Dr Lars Hagberg has the following conflicts: Payment for lecture for infectious specialists and GP:s from Roche, Meda. Dr Magnus Gisslén has the following conflicts: Money paid by BMS, Gilead, Janssen, GSK and Abbott for scientific advisory board and educational lectures. This does not alter the authors' adherence to all the PLOS ONE policies on sharing data and materials.
http://www.ncbi.nlm.nih.gov/pubmed/19433372
1. Bull Cancer. 2009;96 Suppl 1:S35-43. doi: 10.1684/bdc.2008.0774. [Targeting HER pathway in head and neck and thoracic cancers]. [Article in French] Barlesi F(1), Breen D. Author information: (1)Service d'Oncologie Thoracique, Pôle thorax, Faculté de Médecine, Université de la Méditerranée, Hôpital Sainte-Marguerite, Assistance Publique-Hôpitaux de Marseille, Marseille, France. fabrice.barlesi@mail.ap-hm.fr Bronchial and head and neck (HN) cancers share similarities especially regarding the HER pathway. Therapeutic progresses targeting the HER pathway are based on monoclonal antibodies, especially cetuximab, and tyrosine kinase (TK) inhibitors, targeting HER only, as gefitinib and erlotinib, or HER and other receptor(s), as VEGFR for the ZD6474. The results obtained already led to the registration of cetuximab (combined with radiotherapy) for management of locally advanced HN cancers, and the registration of erlotinib (and gefitinib in some countries) for management of non-small-cell lung cancer (NSCLC) in the second or third line setting. Therefore, these first successes led to the development of several drugs including monoclonal antibodies (trastuzumab, panitumumab, matuzumab), TK inhibitors targeting one receptor as well as TK pan-inhibitors (lapatinib, HKI 272, PKI 166, EKB-569, AEE-788), currently assessed through clinical trials worldwide. In the same time, progresses regarding the HER pathway also focused on a better selection of patients who clearly beneficiate from these drugs (EGFR gene mutations, EGFR gene amplification by FISH) allowing the first steps in tailoring anticancer treatments in lung cancer. In conclusion, therapeutic progresses targeting the HER pathway have improve management of HN and NSCLC patients and rise hopes for the future. DOI: 10.1684/bdc.2008.0774 PMID: 19433372 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/17975102
1. Stroke. 2007 Dec;38(12):3205-12. doi: 10.1161/STROKEAHA.107.489351. Epub 2007 Nov 1. Shift analysis versus dichotomization of the modified Rankin scale outcome scores in the NINDS and ECASS-II trials. Savitz SI(1), Lew R, Bluhmki E, Hacke W, Fisher M. Author information: (1)Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA. sean.i.savitz@uth.tmc.edu BACKGROUND AND PURPOSE: The SAINT I trial that showed a significant benefit of the neuroprotectant NXY-059 used a novel outcome for acute ischemic stroke trials: a shift toward good functional outcome on the 7-category modified Rankin scale (mRS). METHODS: We used the Cochran-Mantel-Haenszel shift test to analyze the distribution of the 90-day mRS outcomes in the NINDS and ECASS-II databases and compared the results with a dichotomized mRS outcome by logistic regression (0 to 2 vs 3 to 6, or 0 to 1 vs 2 to 6). We also stratified each dataset based on National Institutes of Health Stroke Scale baseline severity. RESULTS: Each dataset showed a statistically significant shift in the 90-day mRS distributions favoring tissue plasminogen activator (odds ratio, 1.6 for NINDS, 1.3 for ECASS-II). For ECASS-II, larger shift effects appeared in National Institutes of Health Stroke Scale 0 to 6 and 16 to 40 strata. Similarly, the mRS 0 to 2 analysis but not mRS 0 to 1 found similar treatment effects in both datasets (odds ratio, 1.6 for NINDS, 1.5 for ECASS-II) and similar variations in the low and high strata in the ECASS-II trial. NINDS found no significant treatment effects across the strata. After removing the strata at the fringes, the shift test lost significance in both datasets. CONCLUSIONS: Tissue plasminogen activator causes a beneficial shift toward wellness on the mRS in both the NINDS and ECASS-II trials, and ECASS-II would have been a positive trial according to the shift approach. However, the shift effect is not global for all treated patients and does not outperform the dichotomized 0 to 2 outcome. Patients with mild and severe deficits also shifted favorably on the mRS in the ECASS-II trial. DOI: 10.1161/STROKEAHA.107.489351 PMID: 17975102 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/23419732
1. Free Radic Biol Med. 2013 Sep;62:145-156. doi: 10.1016/j.freeradbiomed.2013.01.033. Epub 2013 Feb 16. Nitrone-based therapeutics for neurodegenerative diseases: their use alone or in combination with lanthionines. Floyd RA(1), Castro Faria Neto HC(2), Zimmerman GA(3), Hensley K(4), Towner RA(5). Author information: (1)Experimental Therapeutics, Oklahoma Medical Research Foundation, Oklahoma City, OK 73104, USA. Electronic address: robert-floyd@omrf.ouhsc.edu. (2)Laboratorio de Immunofarmacologia, Instituto Oswaldo Cruz, IOC, Fiocruz, Rio de Janeiro, Brazil. (3)Laboratorio de Immunofarmacologia, Instituto Oswaldo Cruz, IOC, Fiocruz, Rio de Janeiro, Brazil; Department of Medicine, University of Utah School of Medicine, Salt Lake City, Utah, USA. (4)Department of Pathology and Department of Neurosciences, University of Toledo Medical Center, Toledo, OH. (5)Advanced Magnetic Resonance Center, Oklahoma Medical Research Foundation, Oklahoma City, OK 73104, USA. The possibility of free radical reactions occurring in biological processes led to the development and employment of novel methods and techniques focused on determining their existence and importance in normal and pathological conditions. For this reason the use of nitrones for spin trapping free radicals became widespread in the 1970s and 1980s, when surprisingly the first evidence of their potent biological properties was noted. Since then widespread exploration and demonstration of the potent biological properties of phenyl-tert-butylnitrone (PBN) and its derivatives took place in preclinical models of septic shock and then in experimental stroke. The most extensive commercial effort made to capitalize on the potent properties of the PBN-nitrones was for acute ischemic stroke. This occurred during 1993-2006, when the 2,4-disulfonylphenyl PBN derivative, called NXY-059 in the stroke studies, was shown to be safe in humans and was taken all the way through clinical phase 3 trials and then was deemed to be ineffective. As summarized in this review, because of its excellent human safety profile, 2,4-disulfonylphenyl PBN, now called OKN-007 in the cancer studies, was tested as an anti-cancer agent in several preclinical glioma models and shown to be very effective. Based on these studies this compound is now scheduled to enter into early clinical trials for astrocytoma/glioblastoma multiforme this year. The potential use of OKN-007 in combination with neurotropic compounds such as the lanthionine ketamine esters is discussed for glioblastoma multiforme as well as for various other indications leading to dementia, such as aging, septic shock, and malaria infections. There is much more research and development activity ongoing for various indications with the nitrones, alone or in combination with other active compounds, as briefly noted in this review. Copyright © 2013 Elsevier Inc. All rights reserved. DOI: 10.1016/j.freeradbiomed.2013.01.033 PMCID: PMC3715559 PMID: 23419732 [Indexed for MEDLINE] Conflict of interest statement: Conflict of Interest. Some of the authors (RAF,RAT and KH) are inventors on issued or pending US patents and RAF is a co-founder of Otologic Pharmaceutics, INC which has in commercial development a treatment consisting of a nitrone combined with an antioxidant for noise-induced hearing loss.
http://www.ncbi.nlm.nih.gov/pubmed/25686607
1. Nature. 2015 Apr 23;520(7548):558-62. doi: 10.1038/nature14154. Epub 2015 Feb 16. Super-enhancers delineate disease-associated regulatory nodes in T cells. Vahedi G(1), Kanno Y(1), Furumoto Y(2), Jiang K(1), Parker SC(3), Erdos MR(3), Davis SR(4), Roychoudhuri R(4), Restifo NP(4), Gadina M(2), Tang Z(5), Ruan Y(5), Collins FS(3), Sartorelli V(6), O'Shea JJ(1). Author information: (1)Lymphocyte Cell Biology Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, Maryland 20892, USA. (2)Translational Immunology Section, NIAMS, NIH, Bethesda, Maryland 20892, USA. (3)Medical Genomics and Metabolic Genetics Branch, National Human Genome Research Institute, NIH, Bethesda, Maryland 20892, USA. (4)Center for Cancer Research, National Cancer Institute, NIH, Bethesda, Maryland 20892, USA. (5)The Jackson Laboratory for Genomic Medicine and Department of Genetic and Development Biology, University of Connecticut, Farmington, Connecticut 06030, USA. (6)Laboratory of Muscle Stem Cells and Gene Regulation, NIAMS, NIH, Bethesda, Maryland 20892, USA. Enhancers regulate spatiotemporal gene expression and impart cell-specific transcriptional outputs that drive cell identity. Super-enhancers (SEs), also known as stretch-enhancers, are a subset of enhancers especially important for genes associated with cell identity and genetic risk of disease. CD4(+) T cells are critical for host defence and autoimmunity. Here we analysed maps of mouse T-cell SEs as a non-biased means of identifying key regulatory nodes involved in cell specification. We found that cytokines and cytokine receptors were the dominant class of genes exhibiting SE architecture in T cells. Nonetheless, the locus encoding Bach2, a key negative regulator of effector differentiation, emerged as the most prominent T-cell SE, revealing a network in which SE-associated genes critical for T-cell biology are repressed by BACH2. Disease-associated single-nucleotide polymorphisms for immune-mediated disorders, including rheumatoid arthritis, were highly enriched for T-cell SEs versus typical enhancers or SEs in other cell lineages. Intriguingly, treatment of T cells with the Janus kinase (JAK) inhibitor tofacitinib disproportionately altered the expression of rheumatoid arthritis risk genes with SE structures. Together, these results indicate that genes with SE architecture in T cells encompass a variety of cytokines and cytokine receptors but are controlled by a 'guardian' transcription factor, itself endowed with an SE. Thus, enumeration of SEs allows the unbiased determination of key regulatory nodes in T cells, which are preferentially modulated by pharmacological intervention. DOI: 10.1038/nature14154 PMCID: PMC4409450 PMID: 25686607 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/24941067
1. PLoS One. 2014 Jun 18;9(6):e99870. doi: 10.1371/journal.pone.0099870. eCollection 2014. Neurological assessment and its relationship to CSF biomarkers in amateur boxers. Neselius S(1), Brisby H(1), Marcusson J(2), Zetterberg H(3), Blennow K(4), Karlsson T(5). Author information: (1)Department of Orthopaedics, Sahlgrenska University Hospital, Gothenburg, Sweden; Institute for Clinical Sciences, the Sahlgrenska Academy at the University of Gothenburg, Gothenburg, Sweden. (2)Geriatric Section, University Hospital in Linköping, Linköping, Sweden; Institution of Clinical and Experimental Medicine, Linköping University, Linköping, Sweden. (3)Clinical Neurochemistry Laboratory, Sahlgrenska University Hospital, Mölndal, Sweden; Institute of Neuroscience and Physiology, Department of Psychiatry and Neurochemistry, the Sahlgrenska Academy at the University of Gothenburg, Gothenburg, Sweden; UCL Institute of Neurology, Queen Square, London, United Kingdom. (4)Clinical Neurochemistry Laboratory, Sahlgrenska University Hospital, Mölndal, Sweden; Institute of Neuroscience and Physiology, Department of Psychiatry and Neurochemistry, the Sahlgrenska Academy at the University of Gothenburg, Gothenburg, Sweden. (5)Disability Research, Department of Behavioral Sciences and Learning, Linköping University, Linköping, Sweden; Linnaeus Centre HEAD, Linköping University, Linköping, Sweden. BACKGROUND: Mild traumatic brain injury (TBI) or concussion is common in many sports. Today, neuropsychological evaluation is recommended in the monitoring of a concussion and in return-to-play considerations. To investigate the sensitivity of neuropsychological assessment, we tested amateur boxers post bout and compared with controls. Further the relationship between neuropsychological test results and brain injury biomarkers in the cerebrospinal fluid (CSF) were investigated. METHOD: Thirty amateur boxers on high elite level with a minimum of 45 bouts and 25 non-boxing matched controls were included. Memory tests (Rey Osterrieth Complex Figure, Listening Span, Digit Span, Controlled Word Association Test, and computerized testing of episodic memory), tests of processing speed and executive functions (Trail Making, Reaction Time, and Finger Tapping) were performed and related to previously published CSF biomarker results for the axonal injury marker neurofilament light (NFL). RESULTS: The neurological assessment showed no significant differences between boxers and controls, although elevated CSF NFL, as a sign of axonal injury, was detected in about 80% of the boxers 1-6 days post bout. The investigation of the relationship between neuropsychological evaluation and CSF NFL concentrations revealed that boxers with persisting NFL concentration elevation after at least 14 days resting time post bout, had a significantly poorer performance on Trail Making A (p = 0.041) and Simple Reaction Time (p = 0.042) compared to other boxers. CONCLUSION: This is the first study showing traumatic axonal brain injury can be present without measureable cognitive impairment. The repetitive, subconcussive head trauma in amateur boxing causes axonal injury that can be detected with analysis of CSF NFL, but is not sufficient to produce impairment in memory tests, tests of processing speed, or executive functions. The association of prolonged CSF NFL increase in boxers with impairment of processing speed is an interesting observation, which needs to be verified in larger studies. DOI: 10.1371/journal.pone.0099870 PMCID: PMC4062456 PMID: 24941067 [Indexed for MEDLINE] Conflict of interest statement: Competing Interests: The authors have declared that no competing interests exist.
http://www.ncbi.nlm.nih.gov/pubmed/21619633
1. BMC Genomics. 2011 May 27;12:270. doi: 10.1186/1471-2164-12-270. Genomic features and computational identification of human microRNAs under long-range developmental regulation. Sheng Y(1), Previti C. Author information: (1)Computational Biology Unit, Bergen Center for Computational Science, and Sars International Centre for Marine Molecular Biology, University of Bergen, Bergen, 5008, Norway. Ying.Sheng@medisin.uio.no BACKGROUND: Recent functional studies have demonstrated that many microRNAs (miRNAs) are expressed by RNA polymerase II in a specific spatiotemporal manner during the development of organisms and play a key role in cell-lineage decisions and morphogenesis. They are therefore functionally related to a number of key protein coding developmental genes, that form genomic regulatory blocks (GRBs) with arrays of highly conserved non-coding elements (HCNEs) functioning as long-range enhancers that collaboratively regulate the expression of their target genes. Given this functional similarity as well as recent zebrafish transgenesis assays showing that the miR-9 family is indeed regulated by HCNEs with enhancer activity, we hypothesized that this type of miRNA regulation is prevalent. In this paper, we therefore systematically investigate the regulatory landscape around conserved self-transcribed miRNAs (ST miRNAs), with their own known or computationally inferred promoters, by analyzing the hallmarks of GRB target genes. These include not only the density of HCNEs in their vicinity but also the presence of large CpG islands (CGIs) and distinct patterns of histone modification marks associated with developmental genes. RESULTS: Our results show that a subset of the conserved ST miRNAs we studied shares properties similar to those of protein-coding GRB target genes: they are located in regions of significantly higher HCNE/enhancer binding density and are more likely to be associated with CGIs. Furthermore, their putative promoters have both activating as well as silencing histone modification marks during development and differentiation. Based on these results we used both an elevated HCNE density in the genomic vicinity as well as the presence of a bivalent promoter to identify 29 putative GRB target miRNAs/miRNA clusters, over two-thirds of which are known to play a role during development and differentiation. Furthermore these predictions include miRNAs of the miR-9 family, which are the only experimentally verified GRB target miRNAs. CONCLUSIONS: A subset of the conserved miRNA loci we investigated exhibits typical characteristics of GRB target genes, which may partially explain their complex expression profiles during development. DOI: 10.1186/1471-2164-12-270 PMCID: PMC3123655 PMID: 21619633 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/22435031
1. Hippokratia. 2011 Jul;15(3):278-9. Identification of a mutation in the MTM1 gene, associated with X-linked myotubular myopathy, in a Greek family. Fidani L, Karagianni P, Tsakalidis C, Mitsiakos G, Hatziioannidis I, Biancalana V, Nikolaidis N. Erratum in Hippokratia. 2021 Apr-Jun;25(2):99. X-linked myotubular myopathy (XLMTM) is a rare congenital myopathy, usually characterized by severe hypotonia and respiratory insufficiency at birth, in affected, male infants. The disease is causally associated with mutations in the MTM1 gene, coding for phosphatase myotubularin. We report a severe case of XLMTM with a novel mutation, at a donor splicing site (c.1467+1G) previously associated with severe phenotype. The mutation was also identified in the patient's mother, providing an opportunity for sound genetic counseling. PMCID: PMC3306040 PMID: 22435031
http://www.ncbi.nlm.nih.gov/pubmed/15373777
1. J Invest Dermatol. 2004 Oct;123(4):715-32. doi: 10.1111/j.0022-202X.2004.23213.x. Stratum corneum keratin structure, function, and formation: the cubic rod-packing and membrane templating model. Norlén L(1), Al-Amoudi A. Author information: (1)Group of Applied Physics-Biomedical, Department of Physics, University of Geneva, Geneva, Switzerland. lars.norlen@physics.unige.ch Comment in J Invest Dermatol. 2004 Oct;123(4):ix-x. doi: 10.1111/j.0022-202X.2004.23242.x. A new model for stratum corneum keratin structure, function, and formation is presented. The structural and functional part of the model, which hereafter is referred to as "the cubic rod-packing model", postulates that stratum corneum keratin intermediate filaments are arranged according to a cubic-like rod-packing symmetry with or without the presence of an intracellular lipid membrane with cubic-like symmetry enveloping each individual filament. The new model could account for (i) the cryo-electron density pattern of the native corneocyte keratin matrix, (ii) the X-ray diffraction patterns, (iii) the swelling behavior, and (iv) the mechanical properties of mammalian stratum corneum. The morphogenetic part of the model, which hereafter is referred to as "the membrane templating model", postulates the presence in cellular space of a highly dynamic small lattice parameter (<30 nm) membrane structure with cubic-like symmetry, to which keratin is associated. It further proposes that membrane templating, rather than spontaneous self-assembly, is responsible for keratin intermediate filament formation and dynamics. The new model could account for (i) the cryo-electron density patterns of the native keratinocyte cytoplasmic space, (ii) the characteristic features of the keratin network formation process, (iii) the dynamic properties of keratin intermediate filaments, (iv) the close lipid association of keratin, (v) the insolubility in non-denaturating buffers and pronounced polymorphism of keratin assembled in vitro, and (vi) the measured reduction in cell volume and hydration level between the stratum granulosum and stratum corneum. Further, using cryo-transmission electron microscopy on native, fully hydrated, vitreous epidermis we show that the subfilametous keratin electron density pattern consists, both in corneocytes and in viable keratinocytes, of one axial subfilament surrounded by an undetermined number of peripheral subfilaments forming filaments with a diameter of approximately 8 nm. DOI: 10.1111/j.0022-202X.2004.23213.x PMID: 15373777 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/23822165
1. Dis Esophagus. 2014 Apr;27(3):294-302. doi: 10.1111/dote.12100. Epub 2013 Jul 4. CXCR4 heterogeneous expression in esophageal squamous cell cancer and stronger metastatic potential with CXCR4-positive cancer cells. Lu CL(1), Guo J, Gu J, Ge D, Hou YY, Lin ZW, Ding JY. Author information: (1)Department of Thoracic Surgery, Zhongshan Hospital, Fudan University, Shanghai, China. CXCR4 belongs to a family of G protein-coupled cell surface receptors and has been proved to a prognostic marker in a various tumors, including esophageal squamous cell cancer. In this study, we analyzed CXCR4 expression in tumor tissue and metastatic tumor tissues of lymph node by immunohistochemistry. CXCR4 was found to be an independent factor of patients' survival and heterogeneously expressed in tumor tissues. Compared with the primary tumor tissues, the scores of CXCR4 expression were significantly higher in corresponding metastatic tumor tissues of lymph nodes (P < 0.01). It was suggested CXCR4-positive cells were prone to migrate to lymph nodes. In the further experiments in vitro, we confirmed heterogeneous expression of CXCR4 in esophageal squamous cell cancer cell lines (KYSE70, Ec109, and CaES17) by flow cytometry analysis. Meanwhile, two subpopulations were isolated from Ec109 based on CXCR4 membrane expression by fluorescence-activated cell sorting. CXCR4-positive cells showed stronger migration ability in Boyden chamber assay than CXCR4 negative ones (P < 0.01). However, no significant difference of cell proliferation was found between the two subpopulations in colony formation assay (P > 0.05). We concluded that CXCR4 might be a key molecule in esophageal squamous cell cancer metastasis. © 2013 Wiley Periodicals, Inc. and the International Society for Diseases of the Esophagus. DOI: 10.1111/dote.12100 PMID: 23822165 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/8779840
1. Am J Physiol. 1996 Feb;270(2 Pt 2):H638-44. doi: 10.1152/ajpheart.1996.270.2.H638. Regulation of SERCA 2 expression by thyroid hormone in cultured chick embryo cardiomyocytes. Fisher DJ(1), Phillips S, McQuinn T. Author information: (1)Lillie Frank Abercrombie Section of Pediatric Cardiology, Department of Pediatrics, Baylor College of Medicine, Houston, Texas, USA. We investigated the role of thyroid hormone in the physiological perinatal increase in cardiac sarcoplasmic reticulum (SR) Ca(2+)-adenosinetriphosphatase (ATPase) expression. We isolated and cultured the cardiomyocytes in 10(-8) M triiodothyronine (T3) for 48 h and then measured SR Ca(2+)-ATPase mRNA and immunodetectable protein contents as well as SR-dependent 45Ca2+ uptake rate. We also examined the effect of T3 on expression of the same gene in monkey kidney CV-1 cells, which do not express thyroid hormone receptors. T3 increased cardiomyocyte SR Ca2+ pump mRNA content by 289 +/- 35%, and immunodetectable SR Ca2+ pump protein content by 255 +/- 44%, and SR-specific 45Ca2+ uptake rate by 189 +/- 22% (P < 0.01 for each). In contrast, T3 had no significant effect on the total cellular RNA or protein contents in the cardiomyocyte, and there was no effect of T3 on Ca(2+)-ATPase mRNA content in the thyroid hormone receptor-negative CV-1 cells. These data demonstrate that T3 increases expression of the cardiac SR Ca2+ pump, that the effect can be localized to the cardiomyocyte, and that the effect is dependent on thyroid hormone receptors. These data are consistent with pretranslational and possibly transcriptional level effect of thyroid hormone on the cardiac SR Ca2+ pump gene (SERCA 2). The gestation-associated increase in thyroid hormone may be at least partially responsible for the previously demonstrated perinatal increase in cardiac SR Ca2+ pump expression. DOI: 10.1152/ajpheart.1996.270.2.H638 PMID: 8779840 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/24339831
1. Front Genet. 2013 Dec 10;4:283. doi: 10.3389/fgene.2013.00283. eCollection 2013. Circ2Traits: a comprehensive database for circular RNA potentially associated with disease and traits. Ghosal S(1), Das S, Sen R, Basak P, Chakrabarti J. Author information: (1)Computational Biology Group, Theory Department, Indian Association for the Cultivation of Science Kolkata, India. Circular RNAs are new players in regulation of post transcriptional gene expression. Animal genomes express many circular RNAs from diverse genomic locations. A recent study has validated a fairly large number of circular RNAs in human, mouse, and nematode. Circular RNAs play a crucial role in fine tuning the level of miRNA mediated regulation of gene expression by sequestering the miRNAs. Their interaction with disease associated miRNAs indicates that circular RNAs are important for disease regulation. In this paper we studied the potential association of circular RNAs (circRNA) with human diseases in two different ways. Firstly, the interactions of circRNAs with disease associated miRNAs were identified, following which the likelihood of a circRNA being associated with a disease was calculated. For the miRNAs associated with individual diseases, we constructed a network of predicted interactions between the miRNAs and protein coding, long non-coding and circular RNA genes. We carried out gene ontology (GO) enrichment analysis on the set of protein coding genes in the miRNA- circRNA interactome of individual diseases to check the enrichment of genes associated with particular biological processes. Secondly, disease associated SNPs were mapped on circRNA loci, and Argonaute (Ago) interaction sites on circular RNAs were identified. We compiled a database of disease-circRNA association in Circ2Traits (http://gyanxet-beta.com/circdb/), the first comprehensive knowledgebase of potential association of circular RNAs with diseases in human. DOI: 10.3389/fgene.2013.00283 PMCID: PMC3857533 PMID: 24339831
http://www.ncbi.nlm.nih.gov/pubmed/15047890
1. Proc Natl Acad Sci U S A. 2004 Apr 6;101(14):5069-74. doi: 10.1073/pnas.0400913101. Epub 2004 Mar 26. Gene discovery in genetically labeled single dopaminergic neurons of the retina. Gustincich S(1), Contini M, Gariboldi M, Puopolo M, Kadota K, Bono H, LeMieux J, Walsh P, Carninci P, Hayashizaki Y, Okazaki Y, Raviola E. Author information: (1)Department of Neurobiology, Harvard Medical School, 220 Longwood Avenue, Boston, MA 02115, USA. In the retina, dopamine plays a central role in neural adaptation to light. Progress in the study of dopaminergic amacrine (DA) cells has been limited because they are very few (450 in each mouse retina, 0.005% of retinal neurons). Here, we applied transgenic technology, single-cell global mRNA amplification, and cDNA microarray screening to identify transcripts present in DA cells. To profile gene expression in single neurons, we developed a method (SMART7) that combines a PCR-based initial step (switching mechanism at the 5' end of the RNA transcript or SMART) with T7 RNA polymerase amplification. Single-cell targets were synthesized from genetically labeled DA cells to screen the RIKEN 19k mouse cDNA microarrays. Seven hundred ninety-five transcripts were identified in DA cells at a high level of confidence, and expression of the most interesting genes was confirmed by immunocytochemistry. Twenty-one previously undescribed proteins were found in DA cells, including a chloride channel, receptors and other membrane glycoproteins, kinases, transcription factors, and secreted neuroactive molecules. Thirty-eight percent of transcripts were ESTs or coding for hypothetical proteins, suggesting that a large portion of the DA cell proteome is still uncharacterized. Because cryptochrome-1 mRNA was found in DA cells, immunocytochemistry was extended to other components of the circadian clock machinery. This analysis showed that DA cells contain the most common clock-related proteins. DOI: 10.1073/pnas.0400913101 PMCID: PMC387375 PMID: 15047890 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/20683334
1. Eur J Anaesthesiol. 2010 Oct;27(10):874-81. doi: 10.1097/EJA.0b013e32833d56b7. Reversal of rocuronium-induced neuromuscular blockade with sugammadex compared with neostigmine during sevoflurane anaesthesia: results of a randomised, controlled trial. Blobner M(1), Eriksson LI, Scholz J, Motsch J, Della Rocca G, Prins ME. Author information: (1)Klinik für Anästhesiologie der Technischen Universität München, Munich, Germany. Blobner@lrz.tum.de BACKGROUND AND OBJECTIVE: Sugammadex, a modified gamma-cyclodextrin, is a selective relaxant-binding agent designed to reverse the effects of the steroidal neuromuscular blocking agents rocuronium or vecuronium. This study compared the efficacy of sugammadex and neostigmine for reversal of neuromuscular blockade induced by rocuronium for facilitating elective surgery. METHODS: This randomised, multicentre, parallel-group trial included 98 adult patients. Patients received intravenous propofol for induction followed by sevoflurane maintenance anaesthesia. Neuromuscular blockade was monitored using acceleromyography and a train-of-four (TOF) mode of stimulation. Patients were randomly allocated to receive sugammadex 2.0 mg kg(-1) or neostigmine 50 microg kg (-1) (with glycopyrrolate 10 microg kg(-1)) at reappearance of the second response of the TOF (mean 16% twitch height of first response) after the last dose of rocuronium. Safety was evaluated by assessing adverse events, laboratory variables and vital signs. RESULTS: Time to recovery of the TOF ratio of 0.9 after sugammadex compared with neostigmine was significantly shorter (P < 0.0001), being 1.5 versus 18.6 min (geometric means). Predictability of response was greater with sugammadex than neostigmine: with 98% of sugammadex patients versus 11% of neostigmine patients recovering to a TOF ratio of 0.9 within 5 min. There were no clinical events related to residual neuromuscular blockade or reoccurrence of blockade. Serious adverse events were observed in two sugammadex-treated patients and in three neostigmine-treated patients, respectively, but none were considered related to study drugs. CONCLUSION: Sugammadex achieved significantly faster recovery of neuromuscular function after rocuronium to a TOF ratio of 0.9 compared with neostigmine (Clinicaltrials.gov identifier: NCT00451217). DOI: 10.1097/EJA.0b013e32833d56b7 PMID: 20683334 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/17289402
1. J Struct Biol. 2007 Jun;158(3):378-85. doi: 10.1016/j.jsb.2006.12.007. Epub 2006 Dec 28. Dissecting the 3-D structure of vimentin intermediate filaments by cryo-electron tomography. Goldie KN(1), Wedig T, Mitra AK, Aebi U, Herrmann H, Hoenger A. Author information: (1)Structural and Computational Biology Unit, European Molecular Biology Lab, Meyerhofstrasse 1, D-69117 Heidelberg, Germany. Vimentin polymerizes via complex lateral interactions of coiled-coil dimers into long, flexible filaments referred to as intermediate filaments (IFs). Intermediate in diameter between microtubules and microfilaments, IFs constitute the third cytoskeletal filament system of metazoan cells. Here we investigated the molecular basis of the 3-D architecture of vimentin IFs by cryo-electron microscopy (cryo-EM) as well as cryo-electron tomography (Cryo-ET) 3-D reconstruction. We demonstrate that vimentin filaments in cross-section exhibit predominantly a four-stranded protofibrilar organization with a right-handed supertwist with a helical pitch of about 96 nm. Compact filaments imaged by cryo-EM appear surprisingly straight and hence appear very stiff. In addition, IFs exhibited an increased flexibility at sites of partial unraveling. This is in strong contrast to chemically fixed, negatively stained preparations of vimentin filaments that generally exhibit smooth bending without untwisting. At some point along the filament unraveling may be triggered and propagates in a cooperative manner so that long stretches of filaments appear to have unraveled rapidly in a coordinated fashion. DOI: 10.1016/j.jsb.2006.12.007 PMID: 17289402 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/16061665
1. Cancer Res. 2005 Aug 1;65(15):6828-34. doi: 10.1158/0008-5472.CAN-05-1119. Cryptochrome, circadian cycle, cell cycle checkpoints, and cancer. Gauger MA(1), Sancar A. Author information: (1)Department of Biochemistry and Biophysics, University of North Carolina School of Medicine, Chapel Hill, North Carolina 27599, USA. It has been reported that disruption of the circadian clock may lead to increased risk of breast cancer in humans and to a high rate or ionizing radiation-induced tumors and mortality in mice. Cryptochrome 1 and cryptochrome 2 proteins are core components of the mammalian circadian clock and mice mutated in both genes are arrhythmic. We tested Cry1-/- Cry2-/- mice and fibroblasts derived from these mice for radiation-induced cancer and killing and DNA damage checkpoints and killing, respectively. We find that the mutant mice are indistinguishable from the wild-type controls with respect to radiation-induced morbidity and mortality. Similarly, the Cry1-/- Cry2-/-mutant fibroblasts are indistinguishable from the wild-type controls with respect to their sensitivity to ionizing radiation and UV radiation and ionizing radiation-induced DNA damage checkpoint response. Our data suggest that disruption of the circadian clock in itself does not compromise mammalian DNA repair and DNA damage checkpoints and does not predispose mice to spontaneous and ionizing radiation-induced cancers. We conclude that the effect of circadian clock disruption on cellular response to DNA damage and cancer predisposition in mice may depend on the mechanism by which the clock is disrupted. DOI: 10.1158/0008-5472.CAN-05-1119 PMID: 16061665 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/20880963
1. Endocr Rev. 2011 Feb;32(1):64-80. doi: 10.1210/er.2009-0040. Epub 2010 Sep 29. Thyronamines--past, present, and future. Piehl S(1), Hoefig CS, Scanlan TS, Köhrle J. Author information: (1)Institut für Experimentelle Endokrinologie, Charité-Universitätsmedizin Berlin, Charité Campus Virchow-Klinikum (Südring 10), Augustenburger Platz 1, 13353 Berlin, Germany. Thyronamines (TAMs) are a newly identified class of endogenous signaling compounds. Their structure is identical to that of thyroid hormone and deiodinated thyroid hormone derivatives, except that TAMs do not possess a carboxylate group. Despite some initial publications dating back to the 1950s, TAMs did not develop into an independent area of research until 2004, when they were rediscovered as potential ligands to a class of G protein-coupled receptors called trace-amine associated receptors. Since this discovery, two representatives of TAMs, namely 3-iodothyronamine (3-T(1)AM) and thyronamine (T(0)AM), have been detected in vivo. Intraperitoneal or central injection of 3-T(1)AM or T(0)AM into mice, rats, or Djungarian hamsters caused various prompt effects, such as metabolic depression, hypothermia, negative chronotropy, negative inotropy, hyperglycemia, reduction of the respiratory quotient, ketonuria, and reduction of fat mass. Although their physiological function remains elusive, 3-T(1)AM and T(0)AM have already revealed promising therapeutic potential because they represent the only endogenous compounds inducing hypothermia as a prophylactic or acute treatment of stroke and might thus be expected to cause fewer side effects than synthetic compounds. This review article summarizes the still somewhat scattered data on TAMs obtained both recently and more than 20 yr ago to yield a complete and updated picture of the current state of TAM research. DOI: 10.1210/er.2009-0040 PMID: 20880963 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/23737378
1. Laryngoscope. 2013 Aug;123(8):1889-95. doi: 10.1002/lary.23884. Epub 2013 Jun 4. Is there a "July effect" for head and neck cancer surgery? Hennessey PT(1), Francis HW, Gourin CG. Author information: (1)Department of Otolaryngology-Head and Neck Surgery, Johns Hopkins University, Baltimore, Maryland 21287, USA. OBJECTIVES/HYPOTHESIS: A "July effect" of increased complications when new trainees begin residency has been reported widely by the media. We sought to determine the effect of admission month on in-hospital mortality, complications, length of hospitalization, and costs for patients undergoing head and neck cancer (HNCA) surgery. STUDY DESIGN: Retrospective cross-sectional study. METHODS: Discharge data from the Nationwide Inpatient Sample for 48,263 patients who underwent an ablative procedure for a malignant oral cavity, laryngeal, hypopharyngeal, or oropharyngeal neoplasm in 2005 to 2008 were analyzed using cross-tabulations and multivariate regression modeling. RESULTS: There were 3,812 cases admitted in July (8%). July admission was significantly associated with Medicaid (RRR 1.40, P = 0.011) or self-pay payor status (RRR 1.40, P = 0.022), medium hospital bed size (RRR 1.63, P = 0.033) and large hospital bed size (RRR 1.73, P = 0.013). There was no association between July admission and other patient or hospital demographic characteristics. Major procedures and comorbidity were significantly associated with in-hospital death, surgical and medical complications, length of hospitalization, and costs, but no association was found for July admission, July through September discharge, or teaching hospital status and short-term morbidity or mortality. Teaching hospitals and large hospital bed size were predictors of increased length of hospitalization and costs; and private, for profit hospitals were additionally associated with increased costs. No interaction between July admission and teaching hospitals was found for any of the outcome variables studied. CONCLUSIONS: These data do not support evidence of a "July effect" or an increase in morbidity or mortality at teaching hospitals providing HNCA surgical care. © 2013 The American Laryngological, Rhinological and Otological Society, Inc. DOI: 10.1002/lary.23884 PMID: 23737378 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/8389710
1. Eur Heart J. 1993 May;14(5):629-33. doi: 10.1093/eurheartj/14.5.629. Triiodothyronine therapy in open-heart surgery: from hope to disappointment. Teiger E(1), Menasché P, Mansier P, Chevalier B, Lajeunie E, Bloch G, Piwnica A. Author information: (1)Department of Cardiovascular Surgery, Hopital Lariboisière, Paris, France. A controversy persists as to whether cardiopulmonary bypass (CPB) decreases plasma levels of triiodothyronine (T3), thereby justifying peri-operative administration of T3 to improve haemodynamic recovery. To examine the effects of T3 therapy on post-CPB haemodynamics and to determine whether the potential inotropic effects of T3 are mediated by an increase in beta-adrenergic responsiveness, a prospective, randomized, double-blind, placebo-controlled study was performed in 20 patients undergoing cardiac surgery with CPB. T3 or placebo solution (10 patients in each group) was given intravenously at the time of aortic unclamping and 4, 8, 12 and 20 h thereafter. End points included (1) thyroid hormone levels measured by radioimmunoassay (2) standard haemodynamic parameters (3) the density of lymphocyte beta-adrenoceptors measured by a radioligand (125I-iodocyanopindolol) binding technique. Post-CPB values (cross clamp removal) of T3 (pg.ml-1) were not significantly decreased compared with pre-CPB values: 3.3 +/- 0.2 vs 3.1 +/- 0.2 in controls and 3.3 +/- 0.4 vs 3.7 +/- 0.6 in T3-treated patients, respectively. The haemodynamic parameters were no different between the two groups at any postoperative time point. Likewise, density and affinity of lymphocyte beta-adrenoceptors were not significantly different from pre-operative values in either group. Thus, there seems to be no sound justification for a routine use of T3 in patients undergoing open-heart procedures. DOI: 10.1093/eurheartj/14.5.629 PMID: 8389710 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/1415533
1. Am J Physiol. 1992 Sep;263(3 Pt 1):E534-40. doi: 10.1152/ajpendo.1992.263.3.E534. Thyroid hormone effects on cardiac gene expression independent of cardiac growth and protein synthesis. Ojamaa K(1), Samarel AM, Kupfer JM, Hong C, Klein I. Author information: (1)Department of Medicine, North Shore University Hospital, Manhasset, New York 11030. Prior studies have demonstrated the importance of hemodynamic loading in mediating thyroxine (T4)-induced cardiac hypertrophy. Direct cellular effects of thyroid hormone have been implicated in modulating the expression of the myosin heavy chain (MHC) genes and the slow sarcoplasmic reticulum calcium adenosine triphosphatase (SR Ca(2+)-ATPase) gene. In the present report, administration of T4 for 72 h did not stimulate growth of the hemodynamically unloaded heterotopic isograft. The synthetic rates of total cardiac proteins and MHC in the isograft remained significantly lower at 64 and 53% of the respective rates measured simultaneously in the in situ working heart. Although total left ventricle RNA content in the isograft was unchanged by T4, alpha-MHC and SR Ca(2+)-ATPase mRNA concentrations were increased 181 and 208%, respectively, and the previously observed beta-MHC expression was completely prevented. These data indicate that, although T4 requires an increased hemodynamic load to stimulate cardiac protein synthesis, it is capable of directly altering the expression of at least two myocyte-specific genes. Therefore some of the phenotypic alterations observed with thyroid hormone treatment are the result of direct effects of the hormones on specific cardiac genes and independent of changes in cardiac growth. DOI: 10.1152/ajpendo.1992.263.3.E534 PMID: 1415533 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/9781038
1. Eur J Hum Genet. 1998 Jul-Aug;6(4):325-30. doi: 10.1038/sj.ejhg.5200189. Genomic organization of the MTM1 gene implicated in X-linked myotubular myopathy. Laporte J(1), Guiraud-Chaumeil C, Tanner SM, Blondeau F, Hu LJ, Vicaire S, Liechti-Gallati S, Mandel JL. Author information: (1)Institut de Génétique et de Biologie Moléculaire et Cellulaire, CNRS, Illkirch, France. X-linked recessive myotubular myopathy (XLMTM) is a very severe congenital muscular disease characterised by an impaired maturation of muscle fibres, and caused by defects in the MTM1 gene. This gene defines a new family of putative tyrosine phosphatases conserved through evolution. We have determined intronic flanking sequences for all the 15 exons to facilitate the detection of mutations in patients and genetic counselling. We characterised a new polymorphic marker in the immediate vicinity of the gene, which might prove useful for linkage analysis. Sequencing of the TATA-less predicted promoter provides the basis for transcriptional regulatory studies. DOI: 10.1038/sj.ejhg.5200189 PMID: 9781038 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/9309268
1. Pathol Biol (Paris). 1997 Jun;45(6):500-5. Genetics of ageing. Macieira-Coelho A(1). Author information: (1)INSERM Génétique et Vieillissement, Université de Paris VI, Versailles, France. The classical arguments favouring a genetic basis of ageing are reviewed emphasizing the questions that remain unanswered. Genes cannot be the sole genetic determinants of ageing. Mendel's paradigm cannot anymore explain all the results recently obtained. Different aspects of the organization of the genome must also play a role in ageing. The functions of the largest part of the human genome remain unknown. Moreover the different genetic theories of ageing are based on natural selection. However, other paradigms are being proposed that can complement or replace Darwin's paradigm. They are based on the spontaneous organization of complex systems. They must be considered in the reappraisal of the ageing phenomenon. PMID: 9309268 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/22855832
1. Genes Dev. 2012 Aug 1;26(15):1714-28. doi: 10.1101/gad.194209.112. Cfp1 integrates both CpG content and gene activity for accurate H3K4me3 deposition in embryonic stem cells. Clouaire T(1), Webb S, Skene P, Illingworth R, Kerr A, Andrews R, Lee JH, Skalnik D, Bird A. Author information: (1)Wellcome Trust Centre for Cell Biology, University of Edinburgh, United Kingdom. Trimethylation of histone H3 Lys 4 (H3K4me3) is a mark of active and poised promoters. The Set1 complex is responsible for most somatic H3K4me3 and contains the conserved subunit CxxC finger protein 1 (Cfp1), which binds to unmethylated CpGs and links H3K4me3 with CpG islands (CGIs). Here we report that Cfp1 plays unanticipated roles in organizing genome-wide H3K4me3 in embryonic stem cells. Cfp1 deficiency caused two contrasting phenotypes: drastic loss of H3K4me3 at expressed CGI-associated genes, with minimal consequences for transcription, and creation of "ectopic" H3K4me3 peaks at numerous regulatory regions. DNA binding by Cfp1 was dispensable for targeting H3K4me3 to active genes but was required to prevent ectopic H3K4me3 peaks. The presence of ectopic peaks at enhancers often coincided with increased expression of nearby genes. This suggests that CpG targeting prevents "leakage" of H3K4me3 to inappropriate chromatin compartments. Our results demonstrate that Cfp1 is a specificity factor that integrates multiple signals, including promoter CpG content and gene activity, to regulate genome-wide patterns of H3K4me3. DOI: 10.1101/gad.194209.112 PMCID: PMC3418589 PMID: 22855832 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/23395387
1. Cytokine. 2013 Mar;61(3):792-800. doi: 10.1016/j.cyto.2013.01.003. Epub 2013 Feb 8. Differences are evident within the CXCR4-CXCL12 axis between ethnically divergent South African populations. Shalekoff S(1), Schramm DB, Lassaunière R, Picton AC, Tiemessen CT. Author information: (1)Centre for HIV and STIs, National Institute for Communicable Diseases, and Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa. The G-protein-coupled receptor, CXCR4, is highly expressed on a number of cell types, and together with its ligand, CXCL12, plays an important role in immune development and trafficking of cells. CXCR4 promotes tumor growth, angiogenesis and metastasis, and is a prognostic marker in a number of different types of tumors. Additionally, CXCR4 is utilized, together with CD4, for entry of T-tropic HIV viruses. Ethnic differences in incidence and mortality of various cancers, and in the response to highly active antiretroviral treatment (HAART) of HIV-1 infected individuals have been reported. The aim of this study was to establish if differences in the CXCR4-CXCL12 axis exist between ethnically divergent uninfected South Africans. CXCR4 density was significantly higher on CD4(+) and CD8(+) T cells, B cells and CD56(dim) NK cells, and CXCL12 levels lower in Black compared with Caucasian individuals. Furthermore, an inverse correlation was observed between CXCR4 density on CD56(+) and CD3(+) cells and age, only in Black individuals. CXCL12-3'A heterozygosity (AG) found in 28% of Caucasians did not explain the higher plasma levels of CXCL12 compared to Black individuals who were all GG genotypes, suggesting that other factors influence homeostatic levels of CXCL12. In conclusion, this study demonstrates that ethnically divergent populations show clear differences in both CXCR4 density and CXCL12 plasma levels which may influence the course of cancer and HIV-1 infection. Copyright © 2013 Elsevier Ltd. All rights reserved. DOI: 10.1016/j.cyto.2013.01.003 PMID: 23395387 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/18207470
1. Semin Fetal Neonatal Med. 2008 Apr;13(2):57-62. doi: 10.1016/j.siny.2007.11.001. Epub 2008 Jan 22. Recent progress in non-invasive prenatal diagnosis. Hahn S(1), Zhong XY, Holzgreve W. Author information: (1)University Women's Hospital/Department of Biomedicine, University Hospital Basel, Switzerland. shahn@uhbs.ch Although the first finding that fetal cells can enter the maternal circulation was made more than a century ago, it is still unclear if this finding will be translated into a clinically useful diagnostic tool in the foreseeable future. However, significant progress has been made via the analysis of cell-free fetal DNA in maternal plasma/serum and clinical services are now already being offered for the determination of fetal rhesus D status and sex. Currently, however, this technology is really only suited for the analysis of fetal genetic loci completely absent from the maternal genome. The detection of more subtle fetal genetic traits, such as point mutations involved in Mendelian disorders (thalassaemia, cystic fibrosis), is considerably more complex. Preliminary reports indicate that the detection of fetal aneuploidies might be possible using epigenetically modified genes, e.g. maspin on chromosome 18. Additionally, an exiting recent development is that it might be feasible to detect Down syndrome via the quantitative assessment of placentally derived cell-free mRNA of chromosome-21-specific genes such as PLAC4. DOI: 10.1016/j.siny.2007.11.001 PMID: 18207470 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/10802345
1. Neurosci Res. 2000 May;37(1):67-78. doi: 10.1016/s0168-0102(00)00102-4. Characterization of the subventricular zone of the adult human brain: evidence for the involvement of Bcl-2. Bernier PJ(1), Vinet J, Cossette M, Parent A. Author information: (1)Laboratoire de Neurobiologie, Centre de Recherche Université Laval Robert-Giffard, 2601 Chemin de la Canardière, Local F-6500, Beauport, Canada. The subventricular zone (SVZ) is an embryonic remnant that persists and remains mitotically active throughout adulthood. The rodent SVZ harbors neuronal precursors, principally in its anterior part, and generates neuroblasts that migrate tangentially into the olfactory bulb, thus forming the so-called rostral migratory stream. This study aimed at characterizing the SVZ in the human brain. Antibodies raised against the widely used SVZ molecular markers nestin, glial fibrillary acidic protein, beta-tubulin-III and polysialylated neural cell adhesion molecule, have allowed us to characterize in detail a zone similar to the rodent SVZ in humans. Virtually all portions of the lateral ventricle, as well as the ventral (hypothalamic) sector of the third ventricle, displayed immunoreactivity for most of the molecular markers. The midline region of the septum (septal recess) and the ventral portion of the SVZ displayed a particularly intense immunostaining for all SVZ markers. These two regions may represent zones of adult neurogenesis that are unique to primates. Furthermore, the anti-apoptotic protein Bcl-2 was found to be actively synthesized and co-expressed with all the other markers throughout the entire SVZ. This study reveals that a well-developed SVZ exists in the adult human brain and suggests that Bcl-2 might play an important role in the functional organization of such a system. DOI: 10.1016/s0168-0102(00)00102-4 PMID: 10802345 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/22140039
1. Am J Physiol Heart Circ Physiol. 2012 Feb 1;302(3):H621-33. doi: 10.1152/ajpheart.00825.2011. Epub 2011 Dec 2. Altered clock gene expression and vascular smooth muscle diurnal contractile variations in type 2 diabetic db/db mice. Su W(1), Xie Z, Guo Z, Duncan MJ, Lutshumba J, Gong MC. Author information: (1)Departments of Physiology, University of Kentucky Medical Center, Lexington, KY 40536, USA. This study was designed to determine whether the 24-h rhythms of clock gene expression and vascular smooth muscle (VSM) contractile responses are altered in type 2 diabetic db/db mice. Control and db/db mice were euthanized at 6-h intervals throughout the day. The aorta, mesenteric arteries, heart, kidney, and brain were isolated. Clock and target gene mRNA levels were determined by either real-time PCR or in situ hybridization. Isometric contractions were measured in isolated aortic helical strips, and pressor responses to an intravenous injection of vasoconstrictors were determined in vivo using radiotelemetry. We found that the 24-h mRNA rhythms of the following genes were suppressed in db/db mice compared with control mice: the clock genes period homolog 1/2 (Per1/2) and cryptochrome 1/2 (Cry1/2) and their target genes D site albumin promoter-binding protein (Dbp) and peroxisome proliferator-activated receptor-γ (Pparg) in the aorta and mesenteric arteries; Dbp in the heart; Per1, nuclear receptor subfamily 1, group D, member 1 (Rev-erba), and Dbp in the kidney; and Per1 in the suprachiasmatic nucleus. The 24-h contractile variations in response to phenylephrine (α(1)-agonist), ANG II, and high K(+) were significantly altered in the aortas from db/db mice compared with control mice. The diurnal variations of the in vivo pressor responses to phenylephrine and ANG II were lost in db/db mice. Moreover, the 24-h mRNA rhythms of the contraction-related proteins Rho kinase 1/2, PKC-potentiated phosphatase inhibitory protein of 17 kDa, calponin-3, tropomyosin-1/2, and smooth muscle protein 22-α were suppressed in db/db mice compared with control mice. Together, our data demonstrated that the 24-h rhythms of clock gene mRNA, mRNA levels of several contraction-related proteins, and VSM contraction were disrupted in db/db mice, which may contribute to the disruption of their blood pressure circadian rhythm. DOI: 10.1152/ajpheart.00825.2011 PMCID: PMC3353796 PMID: 22140039 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/11470472
1. Cardiovasc Res. 2001 Aug 1;51(2):322-30. doi: 10.1016/s0008-6363(01)00287-5. 3,5,3'-Triiodothyronine deprivation affects phenotype and intracellular [Ca2+]i of human cardiomyocytes in culture. Forini F(1), Paolicchi A, Pizzorusso T, Ratto GM, Saviozzi M, Vanini V, Iervasi G. Author information: (1)Istituto di Fisiologia Clinica C.N.R., via Moruzzi 1, 56100, Pisa, Italy. simona@ifc.cnr.it OBJECTIVE: A decrease in plasma T3 concentration is a frequent finding in patients with heart failure. However, the role of this 'low T3 syndrome' on disease evolution has never been clarified. As phenotypic and functional cardiomyocyte impairments are alterations that correlate with the failing myocardium, we studied the long-term effects of T3 deprivation on human cardiomyocyte structure and calcium handling. METHODS: Atrial cardiomyocytes and myocardial tissue were cultured with or without 3 nM T3. Microscopical examination of structural features was followed by analysis of alpha-sarcomeric actinin and sarcoplasmic reticulum calcium ATP-ase (SERCA-2) content. Calcium handling was studied by [Ca2+](i) imaging. RESULTS: When stimulated with cyclopiazonic acid, a SERCA-2 inhibitor, T3-deprived cardiomyocytes showed significantly faster (P=0.03) and more transient (P=0.04) increases in [Ca(2+)](i) than T3-supplemented cells. Moreover, in the T3-free cultures a significantly lower number of cells (P=0.003) responded to caffeine, a typical activator of sarcoplasmic reticulum Ca(2+)-release channel. T3-deprived cardiomyocytes also presented altered morphology with larger dimensions than T3-supplemented cells (P < 0.0001). Additionally, in T3-deprived samples alpha-sarcomeric actinin and SERCA-2 protein levels were reduced to 65.6 +/- 3% (P < 0.0001) and 74.1 +/- 4% (P=0.005), respectively, when compared with the T3-supplemented group. CONCLUSIONS: Our data show that human cardiomyocyte calcium handling and phenotype are strongly influenced by T3 suggesting important implications of the 'low T3 syndrome' on the progression of heart failure. DOI: 10.1016/s0008-6363(01)00287-5 PMID: 11470472 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/24578770
1. West J Emerg Med. 2014 Feb;15(1):88-93. doi: 10.5811/westjem.2013.10.18123. The July Effect: is emergency department length of stay greater at the beginning of the hospital academic year? Riguzzi C(1), Hern HG(1), Vahidnia F(1), Herring A(1), Alter H(1). Author information: (1)Highland Hospital, Alameda Health System, Oakland, California. INTRODUCTION: There has been concern of increased emergency department (ED) length of stay (LOS) during the months when new residents are orienting to their roles. This so-called "July Effect" has long been thought to increase LOS, and potentially contribute to hospital overcrowding and increased waiting time for patients. The objective of this study is to determine if the average ED LOS at the beginning of the hospital academic year differs for teaching hospitals with residents in the ED, when compared to other months of the year, and as compared to non-teaching hospitals without residents. METHODS: We performed a retrospective analysis of a nationally representative sample of 283,621 ED visits from the National Hospital Ambulatory Medical Care Survey (NHAMCS), from 2001 to 2008. We stratified the sample by proportion of visits seen by a resident, and compared July to the rest of the year, July to June, and July and August to the remainder of the year. We compared LOS for teaching hospitals to non-teaching hospitals. We used bivariate statistics, and multivariable regression modeling to adjust for covariates. RESULTS: Our findings show that at teaching hospitals with residents, there is no significant difference in mean LOS for the month of July (275 minutes) versus the rest of the year (259 min), July and August versus the rest of the year, or July versus June. Non-teaching hospital control samples yielded similar results with no significant difference in LOS for the same time periods. There was a significant difference found in mean LOS at teaching hospitals (260 minutes) as compared to non-teaching hospitals (185 minutes) throughout the year (p<0.0001). CONCLUSION: Teaching hospitals with residents in the ED have slower throughput of patients, no matter what time of year. Thus, the "July Effect" does not appear to a factor in ED LOS. This has implications as overcrowding and patient boarding become more of a concern in our increasingly busy EDs. These results question the need for additional staffing early in the academic year. Teaching hospitals may already institute more robust staffing during this time, preventing any significant increase in LOS. Multiple factors contribute to long stays in the ED. While patients seen by residents stay longer in the ED, there is little variability throughout the academic year. DOI: 10.5811/westjem.2013.10.18123 PMCID: PMC3935791 PMID: 24578770 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/22952936
1. PLoS One. 2012;7(8):e44237. doi: 10.1371/journal.pone.0044237. Epub 2012 Aug 29. Diurnal variation of hepatic antioxidant gene expression in mice. Xu YQ(1), Zhang D, Jin T, Cai DJ, Wu Q, Lu Y, Liu J, Klaassen CD. Author information: (1)Zunyi Medical College, Zunyi, China. BACKGROUND: This study was aimed to examine circadian variations of hepatic antioxidant components, including the Nrf2- pathway, the glutathione (GSH) system, antioxidant enzymes and metallothionein in mouse liver. METHODS AND RESULTS: Adult mice were housed in light- and temperature-controlled facilities for 2 weeks, and livers were collected every 4 h during the 24 h period. Total RNA was isolated, purified, and subjected to real-time RT-PCR analysis. Hepatic mRNA levels of Nrf2, Keap1, Nqo1 and Gclc were higher in the light-phase than the dark-phase, and were female-predominant. Hepatic GSH presented marked circadian fluctuations, along with glutathione S-transferases (GST-α1, GST-µ, GST-π) and glutathione peroxidase (GPx1). The expressions of GPx1, GST-µ and GST-π mRNA were also higher in females. Antioxidant enzymes Cu/Zn superoxide dismutase (Sod1), catalase (CAT), cyclooxygenase-2 (Cox-2) and heme oxygenase-1 (Ho-1) showed circadian rhythms, with higher expressions of Cox-2 and CAT in females. Metallothionein, a small non-enzymatic antioxidant protein, showed dramatic circadian variation in males, but higher expression in females. The circadian variations of the clock gene Brain and Muscle Arnt-like Protein-1(Bmal1), albumin site D-binding protein (Dbp), nuclear receptor Rev-Erbα (Nr1d1), period protein (Per1 and Per2) and cryptochrome 1(Cry1) were in agreement with the literature. Furthermore, acetaminophen hepatotoxicity is more severe when administered in the afternoon when hepatic GSH was lowest. CONCLUSIONS: Circadian variations and gender differences in transcript levels of antioxidant genes exist in mouse liver, which could affect body responses to oxidative stress at different times of the day. DOI: 10.1371/journal.pone.0044237 PMCID: PMC3430632 PMID: 22952936 [Indexed for MEDLINE] Conflict of interest statement: Competing Interests: The authors have declared that no competing interests exist.
http://www.ncbi.nlm.nih.gov/pubmed/20512532
1. J Gen Intern Med. 2010 Aug;25(8):774-9. doi: 10.1007/s11606-010-1356-3. Epub 2010 May 29. A July spike in fatal medication errors: a possible effect of new medical residents. Phillips DP(1), Barker GE. Author information: (1)Department of Sociology, University of California at San Diego, 0533, 9500 Gilman Drive, La Jolla, CA 92093-0533, USA. dphillips@ucsd.edu Comment in J Gen Intern Med. 2010 Aug;25(8):760-1. doi: 10.1007/s11606-010-1421-y. J Gen Intern Med. 2011 Jan;26(1):10; author reply 11. doi: 10.1007/s11606-010-1532-5. BACKGROUND: Each July thousands begin medical residencies and acquire increased responsibility for patient care. Many have suggested that these new medical residents may produce errors and worsen patient outcomes-the so-called "July Effect;" however, we have found no U.S. evidence documenting this effect. OBJECTIVE: Determine whether fatal medication errors spike in July. DESIGN: We examined all U.S. death certificates, 1979-2006 (n = 62,338,584), focusing on medication errors (n = 244,388). We compared the observed number of deaths in July with the number expected, determined by least-squares regression techniques. We compared the July Effect inside versus outside medical institutions. We also compared the July Effect in counties with versus without teaching hospitals. OUTCOME MEASURE: JR = Observed number of July deaths / Expected number of July deaths. RESULTS: Inside medical institutions, in counties containing teaching hospitals, fatal medication errors spiked by 10% in July and in no other month [JR = 1.10 (1.06-1.14)]. In contrast, there was no July spike in counties without teaching hospitals. The greater the concentration of teaching hospitals in a region, the greater the July spike (r = .80; P = .005). These findings held only for medication errors, not for other causes of death. CONCLUSIONS: We found a significant July spike in fatal medication errors inside medical institutions. After assessing competing explanations, we concluded that the July mortality spike results at least partly from changes associated with the arrival of new medical residents. DOI: 10.1007/s11606-010-1356-3 PMCID: PMC2896592 PMID: 20512532 [Indexed for MEDLINE] Conflict of interest statement: None disclosed.
http://www.ncbi.nlm.nih.gov/pubmed/18208360
1. Biol Chem. 2008 Mar;389(3):279-83. doi: 10.1515/BC.2008.033. FoxO transcription factors in oxidative stress response and ageing--a new fork on the way to longevity? Sedding DG(1). Author information: (1)Department of Cardiology, Justus-Liebig University, D-35392 Giessen, Germany. daniel.sedding@innere.med.uni-giessen.de Forkhead box O (FoxO) transcription factors are important downstream targets of the PI3K/Akt signaling pathway and crucial regulators of cell fate. This function of FoxOs relies on their ability to control diverse cellular functions, including proliferation, differentiation, apoptosis, DNA repair, defense against oxidative stress and ageing. FoxOs are regulated by a variety of different growth factors and hormones, and their activity is tightly controlled by post-translational modifications, including phosphorylation, acetylation, ubiquitination and interaction with different proteins and transcription factors. This brief review focuses on the molecular mechanisms, cellular effects and resulting organismal phenotypes generated by differentially regulated FoxO proteins and discusses our current understanding of the role of FoxOs in disease and ageing processes. DOI: 10.1515/BC.2008.033 PMID: 18208360 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/19468253
1. Pathobiology. 2009 May;76(3):136-40. doi: 10.1159/000209391. Epub 2009 May 19. Immunolocalization of DNMT1 and DNMT3a in salivary gland neoplasms. Cavaliéri Gomes C(1), da Silveira e Oliveira C, Santos Pimenta LG, De Marco L, Santiago Gomez R. Author information: (1)School of Dentistry, Pontifícia Universidade Católica de Minas Gerais, Belo Horizonte, Brazil. OBJECTIVE: Salivary gland neoplasms pathogenesis has not been well established. DNA methylation occurs when methyl groups are added to cytosine nucleotides in specific areas of the gene by the enzyme DNA methyltransferase (DNMT). This chemical modification can alter gene expression without altering DNA sequence. While DNMT3a is mostly involved in de novo methylation, DNMT1 acts as a maintenance methyltransferase. We aimed to investigate the immunoexpression of DNMT3a and DNMT1 in minor salivary gland neoplasms, comparing it with normal tissue. MATERIAL: Forty-four formalin-fixed and paraffin-embedded samples of pleomorphic adenoma, adenoid cystic carcinoma, mucoepidermoid carcinoma and polymorphous low-grade adenocarcinoma were included in the study. The DNMT1 and DNMT3a proteins were identified by using a highly sensitive polymer-based system. RESULTS: Positive nuclear and cytoplasmic labeling for DNMT1 was observed in all samples, including the controls. Positive nuclear labeling for DNMT3a was found only in few neoplasms: 1 pleomorphic adenoma (9.0%), 2 adenoid cystic carcinoma (16.6%) and 1 mucoepidermoid (9.0%) cases. CONCLUSION: Our results were not able to demonstrate a clear correlation between DNMT1 and DNMT3a immunoexpression and salivary gland neoplasms development. Copyright 2009 S. Karger AG, Basel. DOI: 10.1159/000209391 PMID: 19468253 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/19778587
1. Neuroscience. 2009 Dec 29;164(4):1531-7. doi: 10.1016/j.neuroscience.2009.09.039. Epub 2009 Sep 22. 5-Lipoxygenase and epigenetic DNA methylation in aging cultures of cerebellar granule cells. Imbesi M(1), Dzitoyeva S, Ng LW, Manev H. Author information: (1)Department of Psychiatry, The Psychiatric Institute,University of Illinois at Chicago, Chicago, IL 60612, USA. 5-Lipoxygenase (5-Lox), an enzyme involved in the metabolism of arachidonic acid participates in the modulation of the proliferation and differentiation of neural stem cells and cerebellar granule cell (CGC) precursors. Since epigenetic mechanisms including DNA methylation regulate 5-LOX expression and have been suggested as possible modulators of stem cell differentiation and aging, using primary cultures of mouse CGC (1, 5, 10, 14, 30 days in vitro; DIV), we studied DNA methylation patterns of the 5-LOX promoter and 5-LOX mRNA levels. We also measured the mRNA and protein content of the DNA methyltransferases DNMT1 and DNMT3a. 5-LOX, DNMT1, and DNMT3a mRNA levels were measured by real-time PCR. We observed that 5-LOX expression and the expression of maintenance DNMT1 is maximal at 1 DIV (proliferating neuronal precursors), whereas the expression of the de novo DNA methyltransferase DNMT3a mRNA increased in aging cultures. We analyzed the methylation status of the 5-LOX promoter using the methylation-sensitive restriction endonucleases AciI, BstUI, HpaII, and HinP1I, which digest unmethylated CpGs while leaving methylated CpGs intact. The 5-LOX DNA methylation increased with the age of the cells. Taken together, our data show that as cultured CGC mature and age in vitro, a decrease in 5-LOX mRNA content is accompanied by an increase in the methylation of the gene DNA. In addition, an increase in DNMT3a but not DNMT1 expression accompanies an increase of 5-LOX methylation during in vitro maturation. DOI: 10.1016/j.neuroscience.2009.09.039 PMCID: PMC2783316 PMID: 19778587 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/9624150
1. J Biol Chem. 1998 Jun 19;273(25):15590-7. doi: 10.1074/jbc.273.25.15590. Type XIII collagen is identified as a plasma membrane protein. Hägg P(1), Rehn M, Huhtala P, Väisänen T, Tamminen M, Pihlajaniemi T. Author information: (1)Collagen Research Unit, Biocenter and Department of Medical Biochemistry, University of Oulu, FIN-90220 Oulu, Finland. The complete primary structure of the mouse type XIII collagen chain was determined by cDNA cloning. Comparison of the mouse amino acid sequences with the previously determined human sequences revealed a high identity of 90%. Surprisingly, the mouse cDNAs extended further in the 5' direction than the previously identified human clones. The 5' sequences contained a new in-frame ATG codon for translation initiation which resulted in elongation of the N-terminal noncollagenous domain by 81 residues. These N-terminal sequences lack a typical signal sequence but include a highly hydrophobic segment that clearly fulfills the criteria for a transmembrane domain. The sequence data thus unexpectedly suggested that type XIII collagen may be located on the plasma membrane, with a short cytosolic N-terminal portion and a long collagenous extracellular portion. These sequence data prompted us to generate antipeptide antibodies against type XIII collagen in order to study the protein and its subcellular location. Western blotting of human tumor HT-1080 cell extract revealed bands of over 180 kDa. These appeared to represent disulfide-bonded multimeric polypeptide forms that resolved upon reduction into 85-95-kDa bands that are likely to represent a mixture of splice forms of monomeric type XIII collagen chains. These chains were shown to contain the predicted N-terminal extension and thus also the putative transmembrane segment. Immunoprecipitation of biotinylated type XIII collagen from surface-labeled HT-1080 cells, subcellular fractionation, and immunofluorescence staining were used to demonstrate that type XIII collagen molecules are indeed located in the plasma membranes of these cells. DOI: 10.1074/jbc.273.25.15590 PMID: 9624150 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/8187356
1. Clin Nephrol. 1994 Mar;41(3):144-9. Effect of enalapril on the microvascular albumin leakage in patients with diabetic microangiopathy and normal or mildly elevated blood pressure. Chagnac A(1), Korzets A, Zevin D, Wender T, Carmon G, Hirsh J, Gafter U, Levi J. Author information: (1)Department of Nephrology, Golda Medical Center, Hasharon Hospital, Petach-Tikva, Israel. The transcapillary escape rate of albumin (TERalb) is often elevated in patients with diabetic microangiopathy. The objective of this study was to examine the effect of enalapril on the TERalb of diabetic patients with albuminuria and normal or mildly elevated blood pressure. Seventeen diabetic patients with diabetic retinopathy, albuminuria, a diastolic blood pressure below 100 mmHg and increased TERalb participated in the study. Blood pressure and TERalb were measured before and after 14 days of therapy with enalapril, 20 mg daily for 14 days. Systolic and diastolic blood pressure fell from 168 +/- 6 to 155 +/- 6 (p < 0.001) and from 87 +/- 2 to 81 +/- 2 mmHg (p < 0.005) respectively. Mean arterial pressure declined from 114 +/- 3 to 105 +/- 3 mmHg (p < 0.0001). The elevated TERalb decreased from 9.5 +/- 0.5 to 7.2 +/- 0.5%/hr (p < 0.005). In the hypertensive subset, systolic, diastolic and mean arterial pressure decreased significantly by 15, 7 and 10 mmHg (p < 0.005, p < 0.005 and p < 0.005 respectively); TERalb decreased from 9.5 +/- 0.6 to 7.3 +/- 0.6 (p < 0.03). In the normotensive subset, arterial pressure remained unchanged and TERalb decreased from 9.0 +/- 0.8 to 6.8 +/- 1.0%/hr (p < 0.03). Plasma fructosamine decreased from 373 +/- 23 to 347 +/- 20 (p < 0.05) in the hypertensive group and remained unchanged in the normotensive patients. No correlation could be demonstrated between variation in TERalb and changes in blood pressure. In conclusion, enalapril decreases microvascular albumin leakage in patients with diabetic microangiopathy and normal or mildly elevated blood pressure. PMID: 8187356 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/20592766
1. Oxid Med Cell Longev. 2009 Jul-Aug;2(3):119-29. doi: 10.4161/oxim.2.3.8916. A fork in the path: Developing therapeutic inroads with FoxO proteins. Maiese K(1), Hou J, Chong ZZ, Shang YC. Author information: (1)Division of Cellular and Molecular Cerebral Ischemia, Wayne State University School of Medicine, Detroit, MI 48201, USA. kmaiese@med.wayne.edu Advances in clinical care for disorders involving any system of the body necessitates novel therapeutic strategies that can focus upon the modulation of cellular proliferation, metabolism, inflammation and longevity. In this respect, members of the mammalian forkhead transcription factors of the O class (FoxOs) that include FoxO1, FoxO3, FoxO4 and FoxO6 are increasingly being recognized as exciting prospects for multiple disorders. These transcription factors govern development, proliferation, survival and longevity during multiple cellular environments that can involve oxidative stress. Furthermore, these transcription factors are closely integrated with several novel signal transduction pathways, such as erythropoietin and Wnt proteins, that may influence the ability of FoxOs to act as a "double-edge sword" to sometimes promote cell survival, but at other times lead to cell injury. Here we discuss the fascinating but complex role of FoxOs during cellular injury and oxidative stress, progenitor cell development, fertility, angiogenesis, cardiovascular function, cellular metabolism and diabetes, cell longevity, immune surveillance and cancer. DOI: 10.4161/oxim.2.3.8916 PMCID: PMC2763237 PMID: 20592766 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/22413869
1. BMC Med. 2012 Mar 13;10:26. doi: 10.1186/1741-7015-10-26. Insufficient maintenance DNA methylation is associated with abnormal embryonic development. Yin LJ(1), Zhang Y, Lv PP, He WH, Wu YT, Liu AX, Ding GL, Dong MY, Qu F, Xu CM, Zhu XM, Huang HF. Author information: (1)Department of Reproductive Endocrinology, Women's Hospital, School of Medicine, Zhejiang University, 1 Xueshi Road, Hangzhou, Zhejiang 310006, China. BACKGROUND: Early pregnancy loss (EPL) is a frustrating clinical problem, whose mechanisms are not completely understood. DNA methylation, which includes maintenance methylation and de novo methylation directed by DNA methyltransferases (DNMTs), is important for embryo development. Abnormal function of these DNMTs may have serious consequences for embryonic development. METHODS: To evaluate the possible involvement of DNA methylation in human EPL, the expression of DNMT proteins and global methylation of DNA were assessed in villous or decidua from EPL patients. The association of maintenance methylation with embryo implantation and development was also examined. RESULTS: We found that DNMT1 and DNMT3A were both expressed in normal human villous and decidua. DNMT1 expression and DNA global methylation levels were significantly down-regulated in villous of EPL. DNMT3A expression was not significantly changed in the EPL group compared to controls in either villous or decidua. We also found that disturbance of maintenance methylation with a DNMT1 inhibitor may result in a decreased global DNA methylation level and impaired embryonic development in the mouse model, and inhibit in vitro embryo attachment to endometrial cells. CONCLUSIONS: Our results demonstrate that defects in DNA maintenance methylation in the embryo, not in the mother, are associated with abnormal embryonic implantation and development. The findings of the current study provide new insights into the etiology of EPL. DOI: 10.1186/1741-7015-10-26 PMCID: PMC3355050 PMID: 22413869 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/8712223
1. Am J Kidney Dis. 1996 Jul;28(1):62-6. doi: 10.1016/s0272-6386(96)90131-6. Accumulation of glycated albumin in end-stage renal failure: evidence for the principle of "physiological microalbuminuria". Donnelly SM(1). Author information: (1)Department of Medicine, Mount Sinai Hospital, University of Toronto, Ontario, Canada. In light of the growing understanding of the toxic effects of glycated albumin and of the preferential excretion of this substance, the excretion of glycated albumin could be considered a physiologic function of the kidney. Furthermore, if the increased load of glycated albumin in diabetic patients results in glycated albumin excretion rates in the range of 20 to 200 microg/min, might this not be considered "physiologic microalbuminuria"? The hypothesis is presented that microalbuminuria composed of glycated albumin is a homeostatic renal function. Although some proteins are glycosylated for their normal physiologic function, many proteins are glycated nonenzymatically according to ambient blood glucose. Albumin is subject to nonenzymatic glycation in all humans, but at increased rates in diabetic patients. Glycated albumin induces changes in the microvasculature and glomerulus that may lead to endothelial dysfunction and diabetic nephropathy, respectively. Renal excretion of glycated albumin is enhanced compared with native albumin. To explore this potential homeostatic function of the kidney, patients with impaired renal function were studied to determine whether glycated albumin accumulates. Plasma levels of glycated albumin were determined in diabetic and nondiabetic patients on hemodialysis. Hemoglobin A1c was used as an index of the rate of nonenzymatic glycation of proteins. Hemoglobin A1c was increased in the diabetic subjects but was normal in the nondiabetic group (7.9% +/- 0.5% v 6.2% +/- 0.2%, respectively; P < 0.01). On the other hand, the glycated albumin was elevated in both groups and was not significantly different between them (1.95% +/- 0.15% in the diabetic patients v 1.75% +/- 0.14% in the nondiabetic patients; P = NS). The results of this study provide the first clinical evidence supporting the hypothesis that the excretion of glycated albumin is a homeostatic renal function. DOI: 10.1016/s0272-6386(96)90131-6 PMID: 8712223 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/18388640
1. Disaster Med Public Health Prep. 2007 Nov;1(2):122-34. doi: 10.1097/DMP.0b013e318158c5fd. Botulism: cause, effects, diagnosis, clinical and laboratory identification, and treatment modalities. Dembek ZF(1), Smith LA, Rusnak JM. Author information: (1)US Army Medical Research Institute of Infectious Diseases, 1425 Porter St, Ft Detrick, MD 21702, USA. zygmunt.dembek@det.amedd.army.mil Botulism is a neuroparalytic disease caused by neurotoxins produced by the bacteria Clostridium botulinum. Botulinum neurotoxins (BoNTs) are among the most potent naturally occurring toxins and are a category A biological threat agent. The 7 toxin serotypes of BoNTs (serotypes A-G) have different toxicities, act through 3 different intracellular protein targets, and exhibit different durations of effect. Botulism may follow ingestion of food contaminated with BoNT, from toxin production of C botulinum present in the intestine or wounds, or from inhalation of aerosolized toxin. Intoxication classically presents as an acute, symmetrical, descending flaccid paralysis. Early diagnosis is important because antitoxin therapy is most effective when administered early. Confirmatory testing of botulism with BoNT assays or C botulinum cultures is time-consuming, and may be insensitive in the diagnosis of inhalational botulism and in as many as 32% of food-borne botulism cases. Therefore, the decision to initiate botulinum antitoxin therapy is primarily based on symptoms and physical examination findings that are consistent with botulism, with support of epidemiological history and electrophysiological testing. Modern clinical practice and antitoxin treatment has reduced botulism mortality rates from approximately 60% to < or =10%. The pentavalent botulinum toxoid is an investigational product and has been used for more than 45 years in at-risk laboratory workers to protect against toxin serotypes A to E. Due to declining immunogenicity and potency of the pentavalent botulinum toxoid, novel vaccine candidates are being developed. DOI: 10.1097/DMP.0b013e318158c5fd PMID: 18388640 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/7796616
1. Cutis. 1995 Apr;55(4):233-6. Acrokeratosis paraneoplastica (Bazex syndrome) with oropharyngeal squamous cell carcinoma. Estrela F(1), Pinto GM, Pinto LM, Afonso A. Author information: (1)Department of Dermatology, Curry Cabral Hospital, Lisboa, Portugal. A 65-year-old white man presented with all the clinical features of acrokeratosis paraneoplastica of Bazex, characterized by violaceous erythema and scaling of the nose, aural helices, fingers, and toes, with keratoderma and severe nail dystrophy. Examination of the patient for possible associated malignancy disclosed an asymptomatic squamous cell carcinoma at the oropharyngeal region. The skin lesions resolved almost completely following radiation therapy of the neoplasm, but the onychodystrophy persisted. This case report illustrates the importance of early recognition of Bazex syndrome. PMID: 7796616 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/24114694
1. Paediatr Drugs. 2014 Feb;16(1):21-8. doi: 10.1007/s40272-013-0051-3. Giant cell tumor of bone in childhood: clinical aspects and novel therapeutic targets. Federman N(1), Brien EW, Narasimhan V, Dry SM, Sodhi M, Chawla SP. Author information: (1)Department of Pediatrics, Hematology/Oncology, Mattel Children's Hospital at University of California, Los Angeles, USA, nfederman@mednet.ucla.edu. Giant cell tumor of bone (GCTB) is a rare primary bone tumor that primarily affects young adults, but can be seen in children. The primary modality of treatment is surgical resection; however, this is not always possible given the location and extent of the neoplasm. Recent developments in the understanding of the underlying molecular pathogenesis of disease have pointed to interactions between the stromal component producing receptor activator of nuclear factor-kappaB (RANK) and RANK-ligand (RANKL) causing the formation of osteoclast-like giant cells that drive bone destruction. The development of a monoclonal humanized antibody to RANKL, denosumab, has been shown to reduce skeletal-related events from osteoporosis and from bony metastases from solid tumors. Recent phase II clinical trials with denosumab in skeletally mature adolescents over age 12 years and adults with GCTB, have shown both safety and efficacy, leading to its accelerated US FDA approval on 13 June 2013. In children who are skeletally immature, safety and efficacy has not been established, and there has been only published anecdotal use. DOI: 10.1007/s40272-013-0051-3 PMID: 24114694 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/14570904
1. J Biol Chem. 2004 Jan 9;279(2):892-900. doi: 10.1074/jbc.M306615200. Epub 2003 Oct 21. Suppression of adriamycin-induced apoptosis by sustained activation of the phosphatidylinositol-3'-OH kinase-Akt pathway. Takeuchi K(1), Ito F. Author information: (1)Department of Biochemistry, Faculty of Pharmaceutical Sciences, Setsunan University, Hirakata, Osaka 573-0101, Japan. The mechanisms by which growth factors trigger signal transduction pathways leading to protection against apoptosis are of great interest. In this study, we investigated the effect of hepatocyte growth factor (HGF/SF) and epidermal growth factor (EGF) on adriamycin (ADR)-induced apoptosis. Treatment of human epithelial MKN74 cells with ADR, a DNA topoisomerase IIalpha inhibitor, caused apoptosis. However, cells pretreated with HGF/SF, but not those pretreated with EGF, were resistant to this apoptosis. The protective effect of HGF/SF against the ADR-induced apoptosis was abolished in the presence of either LY294002, an inhibitor of phosphatidylinositol-3'-OH kinase (PI3-K) or 1L-6-hydroxymethyl-chiro-inositol 2-(R)-2-O-methyl-3-O-octadecylcarbonate, an inhibitor of Akt, thus implicating the activation of PI3-K-Akt signaling in the antiapoptotic action of HGF/SF. Immunoblotting analysis revealed that HGF/SF stimulated the sustained phosphorylation of Akt for several hours but that EGF stimulated the phosphorylation only transiently. Furthermore, ADR-induced activation of caspase-9, a downstream molecule of Akt, was inhibited for at least 24 h after HGF/SF stimulation, but it was not affected by EGF stimulation. Cell-surface biotin-labeling analysis showed that the HGF/SF receptor remained on the cell surface until at least 30 min after HGF/SF addition but that the EGF receptor level on the cell surface was attenuated at an earlier time after EGF addition. These results indicate that HGF/SF, but not EGF, transmitted protective signals against ADR-induced apoptosis by causing sustained activation of the PI3-K-Akt signaling pathway. Furthermore, the difference in antiapoptotic capacity between HGF/SF and EGF is explained, at least in part, by the delayed down-regulation of the HGF/SF receptor. DOI: 10.1074/jbc.M306615200 PMID: 14570904 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/11818019
1. J Biomed Opt. 2002 Jan;7(1):109-22. doi: 10.1117/1.1428292. Morphological spot counting from stacked images for automated analysis of gene copy numbers by fluorescence in situ hybridization. Grigoryan AM(1), Dougherty ER, Kononen J, Bubendorf L, Hostetter G, Kallioniemi O. Author information: (1)University of Texas at San Antonio, College of Engineering, San Antonio, Texas, USA. Fluorescence in situ hybridization (FISH) is a molecular diagnostic technique in which a fluorescent labeled probe hybridizes to a target nucleotide sequence of deoxyribose nucleic acid. Upon excitation, each chromosome containing the target sequence produces a fluorescent signal (spot). Because fluorescent spot counting is tedious and often subjective, automated digital algorithms to count spots are desirable. New technology provides a stack of images on multiple focal planes throughout a tissue sample. Multiple-focal-plane imaging helps overcome the biases and imprecision inherent in single-focal-plane methods. This paper proposes an algorithm for global spot counting in stacked three-dimensional slice FISH images without the necessity of nuclei segmentation. It is designed to work in complex backgrounds, when there are agglomerated nuclei, and in the presence of illumination gradients. It is based on the morphological top-hat transform, which locates intensity spikes on irregular backgrounds. After finding signals in the slice images, the algorithm groups these together to form three-dimensional spots. Filters are employed to separate legitimate spots from fluorescent noise. The algorithm is set in a comprehensive toolbox that provides visualization and analytic facilities. It includes simulation software that allows examination of algorithm performance for various image and algorithm parameter settings, including signal size, signal density, and the number of slices. DOI: 10.1117/1.1428292 PMID: 11818019 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/18450745
1. J Biol Chem. 2008 Jul 11;283(28):19626-35. doi: 10.1074/jbc.M802655200. Epub 2008 May 1. Specificity of the chromodomain Y chromosome family of chromodomains for lysine-methylated ARK(S/T) motifs. Fischle W(1), Franz H, Jacobs SA, Allis CD, Khorasanizadeh S. Author information: (1)Department of Biochemistry and Molecular Genetics, University of Virginia Health System, Charlottesville, Virginia 22908-0733, USA. wfischl@gwdg.de Previous studies have shown two homologous chromodomain modules in the HP1 and Polycomb proteins exhibit discriminatory binding to related methyllysine residues (embedded in ARKS motifs) of the histone H3 tail. Methylated ARK(S/T) motifs have recently been identified in other chromatin factors (e.g. linker histone H1.4 and lysine methyltransferase G9a). These are thought to function as peripheral docking sites for the HP1 chromodomain. In vertebrates, HP1-like chromodomains are also present in the chromodomain Y chromosome (CDY) family of proteins adjacent to a putative catalytic motif. The human genome encodes three CDY family proteins, CDY, CDYL, and CDYL2. These have putative functions ranging from establishment of histone H4 acetylation during spermiogenesis to regulation of transcription co-repressor complexes. To delineate the biochemical functions of the CDY family chromodomains, we analyzed their specificity of methyllysine recognition. We detected substantial differences among these factors. The CDY chromodomain exhibits discriminatory binding to lysine-methylated ARK(S/T) motifs, whereas the CDYL2 chromodomain binds with comparable strength to multiple ARK(S/T) motifs. Interestingly, subtle amino acid changes in the CDYL chromodomain prohibit such binding interactions in vitro and in vivo. However, point mutations can rescue binding. In support of the in vitro binding properties of the chromodomains, the full-length CDY family proteins exhibit substantial variability in chromatin localization. Our studies underscore the significance of subtle sequence differences in a conserved signaling module for diverse epigenetic regulatory pathways. DOI: 10.1074/jbc.M802655200 PMCID: PMC2443675 PMID: 18450745 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/20305384
1. Epigenetics. 2010 Apr;5(3):229-40. doi: 10.4161/epi.5.3.11409. Epub 2010 Apr 1. 5-azacytidine treatment reorganizes genomic histone modification patterns. Komashko VM, Farnham PJ. Methylation of DNA in combination with histone modifications establishes an epigenetic code that ensures the proper control of gene expression. Although DNA methyltransferases have been shown to interact with histone methyltransferases such as EZH2 (which methylates histone H3 on lysine 27) and G9a (which methylates histone H3 on lysine 9), the relationship between DNA methylation and repressive histone marks has not been fully studied. In cancer cells, promoters of genes are often aberrantly methylated. Accordingly, 5-azacytidine (a DNA demethylating drug) is used for treating patients with myelodysplastic syndrome. However, no genome-scale studies of the effects of this drug have been reported. In this work, we report the effects of 5-azacytidine on global gene expression and analyze ~24,000 human promoters using ChIP-chip to determine how 5-azacytidine treatment effects H3K27me3 and H3K9me3 levels. We found that (1) 5-azacytidine treatment results in large changes in gene regulation with distinct functional categories of genes showing increased (e.g. C2H2 zinc finger transcription factors) and decreased (e.g. genes involved in regulation of mitochondria and oxidoreductase activity) levels; (2) most genes that show altered expression are not regulated by promoters that display DNA methylation prior to the treatment; (3) certain gene classes switch their repression mark upon treatment with 5-azacytidine (from H3K27me3 to H3K9me3 and vice versa); and (4) most changes in gene expression are not due to relief of repression mediated by DNA or histone methylation. DOI: 10.4161/epi.5.3.11409 PMID: 20305384 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/20959496
1. Physiol Genomics. 2011 May 1;43(10):534-42. doi: 10.1152/physiolgenomics.00130.2010. Epub 2010 Oct 19. The role of microRNA in modulating myocardial ischemia-reperfusion injury. Ye Y(1), Perez-Polo JR, Qian J, Birnbaum Y. Author information: (1)Department of Biochemistry and Molecular Biology, University of Texas Medical Branch, Galveston, TX 77555, USA. yumye@utmb MicroRNAs (miRNAs) are small (∼22 nt) noncoding single-stranded RNA molecules that downregulate gene expression. Studies have shown that miRNAs control diverse aspects of heart disease, including hypertrophy, remodeling, heart failure, and arrhythmia. Recently, several studies have suggested that miRNAs contribute to ischemia-reperfusion injury by altering key signaling elements, thus making them potential therapeutic targets. By altering the expression of various key elements in cell survival and apoptosis [such as phosphoinositide 3-kinase (PI3K), phosphatase and tensin homolog deleted on chromosome 10 (PTEN), Bcl-2, Mcl-1, heat shock protein (HSP)60, HSP70, HSP20, programmed cell death 4 (Pdcd4), LRRFIP1, Fas ligand (FasL), Sirt-1, etc.], miRNAs alter the response to ischemia-reperfusion injury. Studies using various in vivo, ex vivo, and in vitro models have suggested the possible involvement of miR-1, miR-21, miR-29, miR-92a, miR-133, miR-199a, and miR-320 in ischemia-reperfusion injury and/or remodeling after myocardial infarction. Thus miRNAs could be potential therapeutic targets for the treatment of heart disease. Inhibiting miRNAs by antisense strategies or pharmacological approaches is likely to emerge as an alternative and safe method for conferring short- and intermediate-term protection against ischemia-reperfusion injury. DOI: 10.1152/physiolgenomics.00130.2010 PMID: 20959496 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/8894691
1. Hum Mol Genet. 1996 Oct;5(10):1577-80. doi: 10.1093/hmg/5.10.1577. Mechanism of ret dysfunction by Hirschsprung mutations affecting its extracellular domain. Iwashita T(1), Murakami H, Asai N, Takahashi M. Author information: (1)Department of Pathology, Nagoya University School of Medicine, Japan. Hirschsprung disease (HSCR) is a congenital disorder associated with the absence of intrinsic ganglion cells in the distal gastrointestinal tract. Recently, many missense, nonsense and frameshift mutations of the ret proto-oncogene were found in familial and sporadic cases of HSCR. Consistent with the view that the HSCR phenotype is the result of inactivation of Ret, the missense mutations detected in the tyrosine kinase domain were demonstrated to result in a marked decrease of the kinase activity of Ret. However, the effects of missense mutations found in the extracellular domain remain unknown. We now report that five mutations in the extracellular domain examined inhibit transport of the Ret protein to the plasma membrane. As a consequence, they significantly decreased the transforming activity of Ret with multiple endocrine neoplasia (MEN) 2A mutation for which cell surface expression is required. Our results also demonstrated that long segment HSCR mutations more severely impair transport of Ret to the plasma membrane than a short segment HSCR mutation, suggesting that the level of its cell surface expression may correlate to the HSCR phenotype. DOI: 10.1093/hmg/5.10.1577 PMID: 8894691 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/17825502
1. Drug Alcohol Depend. 2008 Jan 1;92(1-3):3-8. doi: 10.1016/j.drugalcdep.2007.06.017. Epub 2007 Sep 6. Cytisine for smoking cessation: a research agenda. Etter JF(1), Lukas RJ, Benowitz NL, West R, Dresler CM. Author information: (1)Institute of Social and Preventive Medicine, University of Geneva, CMU, Case Postale, CH-1211 Geneva 4, Switzerland. jean-francois.etter@imsp.unige.ch Comment in Drug Alcohol Depend. 2008 Jan 1;92(1-3):1-2. doi: 10.1016/j.drugalcdep.2007.07.010. Cytisine has a molecular structure somewhat similar to that of nicotine and varenicline. The concept for the new smoking cessation drug varenicline was based partly on cytisine. Like varenicline, cytisine is a partial agonist of nicotinic acetylcholine receptors, with high affinity for alpha4beta2 receptors. Cytisine has been used since the 1960s as a smoking cessation drug in Eastern and Central Europe, but has remained largely unnoticed elsewhere. Three placebo-controlled trials, conducted in East and West Germany in the 1960s and 1970s, suggest that cytisine, even with minimal behavioural support, may be effective in aiding smoking cessation. Cytisine tablets are very inexpensive to produce and could be a more affordable treatment than nicotine replacement, bupropion and varenicline. There is however a dearth of scientific research on the properties of cytisine, including safety, abuse liability and efficacy. This paper seeks to identify research priorities for molecular, animal and clinical studies. In particular, new studies are necessary to define the nicotinic receptor interaction profile of cytisine, to establish its pharmacokinetics and pharmacodynamics in humans, to determine whether animals self-administer cytisine, and to ascertain whether cytisine is safe and effective as a smoking cessation drug. Potentially, this research effort, contributing to wider use of an inexpensive drug, could save many lives. DOI: 10.1016/j.drugalcdep.2007.06.017 PMID: 17825502 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/16465592
1. Mamm Genome. 2006 Feb;17(2):119-28. doi: 10.1007/s00335-005-0098-8. Epub 2006 Feb 7. Neuronal expression of the Ccm2 gene in a new mouse model of cerebral cavernous malformations. Plummer NW(1), Squire TL, Srinivasan S, Huang E, Zawistowski JS, Matsunami H, Hale LP, Marchuk DA. Author information: (1)Department of Molecular Genetics and Microbiology, Duke University Medical Center, Durham, North Carolina 27710, USA. Cerebral cavernous malformations are vascular defects of the central nervous system consisting of clusters of dilated vessels that are subject to frequent hemorrhaging. The genes mutated in three forms of autosomal dominant cerebral cavernous malformations have been cloned, but it remains unclear which cell type is ultimately responsible for the lesion. In this article we describe mice with a gene trap insertion in the Ccm2 gene. Consistent with the human phenotype, heterozygous animals develop cerebral vascular malformations, although penetrance is low. Beta-galactosidase activity in heterozygous brain and in situ hybridization in wild-type brain revealed Ccm2 expression in neurons and choroid plexus but not in vascular endothelium of small vessels in the brain. The expression pattern of Ccm2 is similar to that of the Ccm1 gene and its interacting protein ICAP1 (Itgb1bp1). These data suggest that cerebral cavernous malformations arise as a result of defects in the neural parenchyma surrounding the vascular endothelial cells in the brain. DOI: 10.1007/s00335-005-0098-8 PMID: 16465592 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/23866358
1. Prescrire Int. 2013 Jun;22(139):155-9. Bleeding with dabigatran, rivaroxaban, apixaban. No antidote, and little clinical experience. [No authors listed] Dabigatran, rivaroxaban and apixaban are oral anticoagulants used to prevent or treat thrombosis in a variety of situations. Like all anticoagulants, these drugs can provoke bleeding. How should patients be managed if bleeding occurs during dabigatran, rivaroxaban or apixaban therapy? How can the risk of bleeding be reduced in patients who require surgery or other invasive procedures? To answer these questions, we reviewed the available literature, using the standard Prescrire methodology. In clinical trials, warfarin, enoxaparin, dabigatran, rivaroxaban and apixaban were associated with a similar frequency of severe bleeding. Numerous reports of severe bleeding associated with dabigatran have been recorded since this drug was first marketed. Some situations are associated with a particularly high bleeding risk, including: even mild renal failure, advanced age, extremes in body weight and drug-drug interactions, particularly with antiplatelet agents (including aspirin), nonsteroidal antiinflammatory drugs, and many drugs used in cardiovascular indications. In patients treated with dabigatran, rivaroxaban or apixaban, changes in the INR (international normalised ratio) and activated partial thromboplastin time (aPTT) do not correlate with the dose. In early 2013, there is still no routine coagulation test suitable for monitoring these patients; specific tests are only available in specialised laboratories. In early 2013 there is no antidote for dabigatran, rivaroxaban or apixaban, nor any specific treatment with proven efficacy for severe bleeding linked to these drugs. Recommendations on the management of bleeding in this setting are based mainly on pharmacological parameters and on scarce experimen-Haemodialysis reduces the plasma concentration of dabigatran, while rivaroxaban and apixaban cannot be eliminated by dialysis. Prothrombin complex concentrates and recombinant activated factor VII seem to have little or no efficacy, and they carry a poorly documented risk of thrombosis. For patients undergoing surgery or other invasive procedures, clinical practice guidelines are primarily based on pharmacokinetic parameters and on extrapolation of data on vitamin K antagonists. The decision on whether or not to discontinue anticoagulation before the procedure mainly depends on the likely risk of bleeding. In patients at high risk of thrombosis, heparin can be proposed when the anticoagulant is withdrawn. In early 2013, difficulties in the management of bleeding and of situations in which there is a risk of bleeding weigh heavily in the balance of potential harm versus potential benefit of dabigatran, rivaroxaban and apixaban. When an oral anticoagulant is required, it is best to choose warfarin, a vitamin K antagonist, and the drug with which we have the most experience, except in those rare situations in which the INR cannot be maintained within the therapeutic range. PMID: 23866358 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/20219102
1. Mol Cancer. 2010 Mar 10;9:56. doi: 10.1186/1476-4598-9-56. Specific effects of bortezomib against experimental malignant pleural effusion: a preclinical study. Psallidas I(1), Karabela SP, Moschos C, Sherrill TP, Kollintza A, Magkouta S, Theodoropoulou P, Roussos C, Blackwell TS, Kalomenidis I, Stathopoulos GT. Author information: (1)Applied Biomedical Research & Training Center "Marianthi Simou", Department of Critical Care & Pulmonary Services, General Hospital "Evangelismos", National and Kapodistrian University of Athens, Greece. BACKGROUND: We have previously shown that nuclear factor (NF)-kappaB activation of mouse Lewis lung carcinoma (LLC) specifically promotes the induction of malignant pleural effusions (MPE) by these cells. In the present studies we hypothesized that treatment of immunocompetent mice with bortezomib tailored to inhibit cancer cell NF-kappaB activation and not proliferation specifically inhibits MPE formation by LLC cells. RESULTS: Treatment of LLC cells with low concentrations of bortezomib (100 ng/ml) inhibited NF-kappaB activation and NF-kappaB-dependent transcription, but not cellular proliferation. Bortezomib treatment of immunocompetent C57BL/6 mice bearing LLC-induced subcutaneous tumors and MPEs significantly blocked tumor-specific NF-kappaB activation. However, bortezomib treatment did not impair subcutaneous LLC tumor growth, but was effective in limiting LLC-induced MPE. This specific effect was evidenced by significant reductions in effusion accumulation and the associated mortality and was observed with both preventive (beginning before MPE formation) and therapeutic (beginning after MPE establishment) bortezomib treatment. The favorable impact of bortezomib on MPE was associated with suppression of cardinal MPE-associated phenomena, such as inflammation, vascular hyperpermeability, and angiogenesis. In this regard, therapeutic bortezomib treatment had identical favorable results on MPE compared with preventive treatment, indicating that the drug specifically counteracts effusion formation. CONCLUSIONS: These studies indicate that proteasome inhibition tailored to block NF-kappaB activation of lung adenocarcinoma specifically targets the effusion-inducing phenotype of this tumor. Although the drug has limited activity against advanced solid lung cancer, it may prove beneficial for patients with MPE. DOI: 10.1186/1476-4598-9-56 PMCID: PMC2841124 PMID: 20219102 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/17212618
1. Acta Neurol Scand. 2007 Feb;115(2):129-31. doi: 10.1111/j.1600-0404.2006.00727.x. Motor neuron disease recovery associated with IV ceftriaxone and anti-Babesia therapy. Harvey WT(1), Martz D. Author information: (1)Rocky Mountain Chronic Disease Specialists, L.L.C., North Circle Drive, Colorado Springs, CO 80909, USA. wth928@aol.com Comment in Acta Neurol Scand. 2008 Mar;117(3):217. doi: 10.1111/j.1600-0404.2007.00952.x. This report summarizes what we believe to be the first verifiable case of a significant and progressive motor neuron disease (MND) consistent with amyotrophic lateral sclerosis that resolved during treatment with i.v. ceftriaxone plus oral atovaquone and mefloquine. The rationale for use of these antibiotics was (i) positive testing for Borrelia burgdorferi and (ii) red blood cell ring forms consistent with Babesia species infection. The patient has continued to be free of MND signs and symptoms for 15 months, although some symptoms consistent with disseminated Borreliosis remain. DOI: 10.1111/j.1600-0404.2006.00727.x PMID: 17212618 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/22527616
1. Pituitary. 2012 Dec;15 Suppl 1:S61-7. doi: 10.1007/s11102-012-0391-y. Genetic analysis in a patient presenting with meningioma and familial isolated pituitary adenoma (FIPA) reveals selective involvement of the R81X mutation of the AIP gene in the pathogenesis of the pituitary tumor. Guaraldi F(1), Corazzini V, Gallia GL, Grottoli S, Stals K, Dalantaeva N, Frohman LA, Korbonits M, Salvatori R. Author information: (1)Division of Endocrinology, Department of Internal Medicine, University of Turin, 10126, Turin, Italy. Familial isolated pituitary adenoma (FIPA), defined as the occurrence of at least two cases of pituitary adenoma in a family that does not exhibit features of syndromic diseases, such as Carney complex or Multiple Endocrine Neoplasia type 1 or 4, is a rare autosomal dominant disease with low penetrance. About 20 % of the families with FIPA harbor inactivating mutation in aryl hydrocarbon receptor-interacting protein gene (AIP) associated with loss of heterozygosity of the same genetic locus (11q13) in the tumor. Rarely different types of extra-pituitary tumors have been described in the setting of AIP mutation-positive FIPA. We present the case of a patient who was diagnosed with acromegaly due to the AIP mutation c.241C>T (p.R81X) at the age of 34 years, and treated by transsphenoidal surgery. At the age of 43 years she was diagnosed with a meningioma, and at age 46 had recurrence of the somatotropinoma. Genetic studies demonstrated loss of the normal allele (by sequencing and microsatellite analysis) in DNA from the pituitary adenoma but not from the meningioma, suggesting a selective involvement of AIP mutation in the pathogenesis of the pituitary adenoma, and a casual association with the meningioma. Further investigations are required to define the exact role of AIP in non-pituitary tumorigenesis. DOI: 10.1007/s11102-012-0391-y PMID: 22527616 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/16083281
1. J Proteome Res. 2005 Jul-Aug;4(4):1310-7. doi: 10.1021/pr050028f. Quantitative protein expression analysis of CLL B cells from mutated and unmutated IgV(H) subgroups using acid-cleavable isotope-coded affinity tag reagents. Barnidge DR(1), Jelinek DF, Muddiman DC, Kay NE. Author information: (1)Mayo Proteomics Research Center, Department of Biochemistry and Molecular Biology, Mayo Clinic College of Medicine, Rochester, MN 55905, USA. barnidge.david@mayo.edu Relative protein expression levels were compared in leukemic B cells from two patients with chronic lymphocytic leukemia (CLL) having either mutated (M-CLL) or unmutated (UM-CLL) immunoglobulin variable heavy chain genes (IgV(H)). Cells were separated into cytosol and membrane protein fractions then labeled with acid-cleavable ICAT reagents (cICAT). Labeled proteins were digested with trypsin then subjected to SCX and affinity chromatography followed by LC-ESI-MS/MS analysis on a linear ion trap mass spectrometer. A total of 9 proteins from the cytosol fraction and 4 from the membrane fraction showed a 3-fold or greater difference between M-CLL and UM-CLL and a subset of these were examined by Western blot where results concurred with cICAT abundance ratios. The abundance of one of the proteins in particular, the mitochondrial membrane protein cytochrome c oxidase subunit COX G was examined in 6 M-CLL and 6 UM-CLL patients using western blot and results showed significantly greater levels (P < 0.001) in M-CLL patients vs UM-CLL patients. These results demonstrate that stable isotope labeling and mass spectrometry can complement 2D gel electrophoresis and gene microarray technologies for identifying putative and perhaps unique prognostic markers in CLL. DOI: 10.1021/pr050028f PMCID: PMC1403294 PMID: 16083281 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/16409643
1. Retrovirology. 2006 Jan 13;3:5. doi: 10.1186/1742-4690-3-5. SUV39H1 interacts with HTLV-1 Tax and abrogates Tax transactivation of HTLV-1 LTR. Kamoi K(1), Yamamoto K, Misawa A, Miyake A, Ishida T, Tanaka Y, Mochizuki M, Watanabe T. Author information: (1)Department of Medical Genome Sciences, Graduate School of Frontier Sciences, The University of Tokyo, 4-6-1 Shirokanedai, Tokyo 108-8639, Japan. koju1030@ims.u-tokyo.ac.jp BACKGROUND: Tax is the oncoprotein of HTLV-1 which deregulates signal transduction pathways, transcription of genes and cell cycle regulation of host cells. Transacting function of Tax is mainly mediated by its protein-protein interactions with host cellular factors. As to Tax-mediated regulation of gene expression of HTLV-1 and cellular genes, Tax was shown to regulate histone acetylation through its physical interaction with histone acetylases and deacetylases. However, functional interaction of Tax with histone methyltransferases (HMTase) has not been studied. Here we examined the ability of Tax to interact with a histone methyltransferase SUV39H1 that methylates histone H3 lysine 9 (H3K9) and represses transcription of genes, and studied the functional effects of the interaction on HTLV-1 gene expression. RESULTS: Tax was shown to interact with SUV39H1 in vitro, and the interaction is largely dependent on the C-terminal half of SUV39H1 containing the SET domain. Tax does not affect the methyltransferase activity of SUV39H1 but tethers SUV39H1 to a Tax containing complex in the nuclei. In reporter gene assays, co-expression of SUV39H1 represses Tax transactivation of HTLV-1 LTR promoter activity, which was dependent on the methyltransferase activity of SUV39H1. Furthermore, SUV39H1 expression is induced along with Tax in JPX9 cells. Chromatin immunoprecipitation (ChIP) analysis shows localization of SUV39H1 on the LTR after Tax induction, but not in the absence of Tax induction, in JPX9 transformants retaining HTLV-1-Luc plasmid. Immunoblotting shows higher levels of SUV39H1 expression in HTLV-1 transformed and latently infected cell lines. CONCLUSION: Our study revealed for the first time the interaction between Tax and SUV39H1 and apparent tethering of SUV39H1 by Tax to the HTLV-1 LTR. It is speculated that Tax-mediated tethering of SUV39H1 to the LTR and induction of the repressive histone modification on the chromatin through H3 K9 methylation may be the basis for the dose-dependent repression of Tax transactivation of LTR by SUV39H1. Tax-induced SUV39H1 expression, Tax-SUV39H1 interaction and tethering to the LTR may provide a support for an idea that the above sequence of events may form a negative feedback loop that self-limits HTLV-1 viral gene expression in infected cells. DOI: 10.1186/1742-4690-3-5 PMCID: PMC1363732 PMID: 16409643 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/2358611
1. J Am Coll Cardiol. 1990 Jul;16(1):91-5. doi: 10.1016/0735-1097(90)90462-x. Altered thyroid hormone metabolism in advanced heart failure. Hamilton MA(1), Stevenson LW, Luu M, Walden JA. Author information: (1)Division of Cardiology, University of California, Los Angeles School of Medicine 90024-1679. To determine the prevalence and significance of abnormal thyroid hormone metabolism in congestive heart failure, free thyroxine (T4) index, free triiodothyronine (T3) index, reverse T3 and thyrotropin levels were obtained in 84 hospitalized patients with chronic advanced heart failure. Free T4 index was normal in all patients. Free T3 index was reduced or reverse T3 elevated, or both, leading to a low free T3 index/reverse T3 ratio in 49 (58%) of the 84 patients. A low free T3 index/reverse T3 ratio was associated with higher right atrial, pulmonary artery and pulmonary capillary wedge pressures and lower ejection fraction, cardiac index, serum sodium, albumin and total lymphocyte count. In multivariate analysis, the free T3 index/reverse T3 ratio was the only independent predictor of poor 6 week outcome (p less than 0.001); the actuarial 1 year survival rate was 100% for patients with a normal ratio and only 37% for those with a low ratio (p less than 0.0001). A low free T3 index/reverse T3 ratio is associated with poor ventricular function and nutritional status and is the strongest predictor yet identified for short-term outcome in patients with advanced heart failure. DOI: 10.1016/0735-1097(90)90462-x PMID: 2358611 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/25619277
1. FEBS J. 2015 Apr;282(7):1225-41. doi: 10.1111/febs.13211. Epub 2015 Feb 13. How pyridoxal 5'-phosphate differentially regulates human cytosolic and mitochondrial serine hydroxymethyltransferase oligomeric state. Giardina G(1), Brunotti P, Fiascarelli A, Cicalini A, Costa MG, Buckle AM, di Salvo ML, Giorgi A, Marani M, Paone A, Rinaldo S, Paiardini A, Contestabile R, Cutruzzolà F. Author information: (1)Dipartimento di Scienze Biochimiche 'A. Rossi Fanelli', Sapienza Università di Roma, Italy. Adaptive metabolic reprogramming gives cancer cells a proliferative advantage. Tumour cells extensively use glycolysis to sustain anabolism and produce serine, which not only refuels the one-carbon units necessary for the synthesis of nucleotide precursors and for DNA methylation, but also affects the cellular redox homeostasis. Given its central role in serine metabolism, serine hydroxymethyltransferase (SHMT), a pyridoxal 5'-phosphate (PLP)-dependent enzyme, is an attractive target for tumour chemotherapy. In humans, the cytosolic isoform (SHMT1) and the mitochondrial isoform (SHMT2) have distinct cellular roles, but high sequence identity and comparable catalytic properties, which may complicate development of successful therapeutic strategies. Here, we investigated how binding of the cofactor PLP controls the oligomeric state of the human isoforms. The fact that eukaryotic SHMTs are tetrameric proteins while bacterial SHMTs function as dimers may suggest that the quaternary assembly in eukaryotes provides an advantage to fine-tune SHMT function and differentially regulate intertwined metabolic fluxes, and may provide a tool to address the specificity problem. We determined the crystal structure of SHMT2, and compared it to the apo-enzyme structure, showing that PLP binding triggers a disorder-to-order transition accompanied by a large rigid-body movement of the two cofactor-binding domains. Moreover, we demonstrated that SHMT1 exists in solution as a tetramer, both in the absence and presence of PLP, while SHMT2 undergoes a dimer-to-tetramer transition upon PLP binding. These findings indicate an unexpected structural difference between the two human SHMT isoforms, which opens new perspectives for understanding their differing behaviours, roles or regulation mechanisms in response to PLP availability in vivo. © 2015 FEBS. DOI: 10.1111/febs.13211 PMID: 25619277 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/19689438
1. J Am Acad Nurse Pract. 2009 Aug;21(8):423-9. doi: 10.1111/j.1745-7599.2009.00424.x. Burning mouth syndrome: Identification, diagnosis, and treatment. Rivinius C(1). Author information: (1)Jacobson Memorial Hospital Care Center, Inc., Elgin, North Dakota 58533, USA. crivinius2@yahoo.com PURPOSE: To provide an overview of burning mouth syndrome (BMS), describe the role of the clinician when a patient presents with the burning mouth complaint, offer guidance in differentiating the cause of the complaint, and identify potential treatment options for the patient suffering from BMS. DATA SOURCES: A search of MD Consult, Medline, and EBSCO Host Research Databases with the terms "burning mouth" and "BMS." CONCLUSIONS: BMS is a common, chronic disorder of unknown etiology with no underlying or systemic causes or oral signs identified. It affects more than 1 million people in the United States, predominantly postmenopausal women. Despite the common nature of the disorder, it is often misunderstood. Palliative treatment, education, and support should be offered to the patient with idiopathic BMS. A variety of treatment options exist, including benzodiazepines, tricyclic antidepressants, anticonvulsants, alpha-lipoic acid, topical capsaicin, and cognitive therapy can be added to the medication regimen for greater benefit. IMPLICATIONS FOR PRACTICE: The role of the clinician is to obtain a meticulous history and physical examination of the patient, order relevant diagnostic tests, and rule out treatable conditions that may be causing the burning mouth symptom. If secondary causes of BMS are ruled out, the clinician should present treatment options to the patient and consider referral to specialists as necessary. A combination of medications may be more effective than a single medication. DOI: 10.1111/j.1745-7599.2009.00424.x PMID: 19689438 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/15249207
1. Biochem Biophys Res Commun. 2004 Aug 6;320(4):1133-8. doi: 10.1016/j.bbrc.2004.06.066. Blockage of RNA polymerase II at a cyclobutane pyrimidine dimer and 6-4 photoproduct. Mei Kwei JS(1), Kuraoka I, Horibata K, Ubukata M, Kobatake E, Iwai S, Handa H, Tanaka K. Author information: (1)Graduate School of Frontier Biosciences, Osaka University, 1-3 Yamadaoka, Suita, Osaka 565-0871, Japan. The blockage of transcription elongation by RNA polymerase II (pol II) at a DNA damage site on the transcribed strand triggers a transcription-coupled DNA repair (TCR), which rapidly removes DNA damage on the transcribed strand of the expressed gene and allows the resumption of transcription. To analyze the effect of UV-induced DNA damage on transcription elongation, an in vitro transcription elongation system using pol II and oligo(dC)-tailed templates containing a cyclobutane pyrimidine dimer (CPD) or 6-4 photoproduct (6-4PP) at a specific site was employed. The results showed that pol II incorporated nucleotides opposite the CPD and 6-4PP and then stalled. Pol II formed a stable ternary complex consisting of pol II, the DNA damage template, and the nascent transcript. Furthermore, atomic force microscopy imaging revealed that pol II stalled at the damaged region. These findings may provide the basis for analysis of the initiation step of TCR. DOI: 10.1016/j.bbrc.2004.06.066 PMID: 15249207 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/18326497
1. J Biol Chem. 2008 May 9;283(19):13116-23. doi: 10.1074/jbc.M707697200. Epub 2008 Mar 7. Mechanism of ceftriaxone induction of excitatory amino acid transporter-2 expression and glutamate uptake in primary human astrocytes. Lee SG(1), Su ZZ, Emdad L, Gupta P, Sarkar D, Borjabad A, Volsky DJ, Fisher PB. Author information: (1)Department of Urology, Neurosurgery, and Pathology, Herbert Irving Comprehensive Cancer Center, Columbia University Medical Center, New York, New York 10032, USA. Glutamate is an essential neurotransmitter regulating brain functions. Excitatory amino acid transporter (EAAT)-2 is one of the major glutamate transporters primarily expressed in astroglial cells. Dysfunction of EAAT2 is implicated in acute and chronic neurological disorders, including stroke/ischemia, temporal lobe epilepsy, amyotrophic lateral sclerosis, Alzheimer disease, human immunodeficiency virus 1-associated dementia, and growth of malignant gliomas. Ceftriaxone, one of the beta-lactam antibiotics, is a stimulator of EAAT2 expression with neuroprotective effects in both in vitro and in vivo models based in part on its ability to inhibit neuronal cell death by glutamate excitotoxicity. Based on this consideration and its lack of toxicity, ceftriaxone has potential to manipulate glutamate transmission and ameliorate neurotoxicity. We investigated the mechanism by which ceftriaxone enhances EAAT2 expression in primary human fetal astrocytes (PHFA). Ceftriaxone elevated EAAT2 transcription in PHFA through the nuclear factor-kappaB (NF-kappaB) signaling pathway. The antibiotic promoted nuclear translocation of p65 and activation of NF-kappaB. The specific NF-kappaB binding site at the -272 position of the EAAT2 promoter was responsible for ceftriaxone-mediated EAAT2 induction. In addition, ceftriaxone increased glutamate uptake, a primary function of EAAT2, and EAAT2 small interference RNA completely inhibited ceftriaxone-induced glutamate uptake activity in PHFA. Taken together, our data indicate that ceftriaxone is a potent modulator of glutamate transport in PHFA through NF-kappaB-mediated EAAT2 promoter activation. These findings suggest a mechanism for ceftriaxone modulation of glutamate transport and for its potential effects on ameliorating specific neurodegenerative diseases through modulation of extracellular glutamate. DOI: 10.1074/jbc.M707697200 PMCID: PMC2442320 PMID: 18326497 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/8898827
1. Stroke. 1996 Nov;27(11):2131-5. doi: 10.1161/01.str.27.11.2131. Moyamoya and Down syndrome. Clinical and radiological features. Cramer SC(1), Robertson RL, Dooling EC, Scott RM. Author information: (1)Neurology Service, Massachusetts General Hospital, Boston 02114, USA. BACKGROUND AND PURPOSE: Moyamoya disease is a chronic occlusive cerebrovascular disorder characterized by progressive stenosis of the supraclinoid internal carotid artery, with the secondary development of enlarged basal collateral vessels. It may occur as a primary disease or as a syndrome in association with a variety of conditions, and its pathogenesis remains unexplained. There are relatively few reports describing the occurrence of moyamoya in Down syndrome. The aim of this study is to describe the clinical and radiological features of moyamoya syndrome associated with Down syndrome (MM-DS) and to explore theories of moyamoya pathogenesis in these patients. METHODS: Seven children with MM-DS underwent brain imaging, transfemoral angiography, and serial neurological exams. Neurological deficits, poststroke recovery, radiographic infarct characteristics, and angiographic abnormalities were reviewed. RESULTS: The clinical and radiological features of primary moyamoya disease overlap with those of MM-DS. Hemiplegia and aphasia were the most common presentations. Motor recovery was excellent in five of seven cases. Cerebral infarcts were superficial or deep and can occur in a watershed distribution. Angiography demonstrated involvement of the internal carotid artery and its branches bilaterally in all seven cases and the posterior cerebral arteries in four cases. CONCLUSIONS: The clinical and radiological features of MM-DS overlap with primary moyamoya disease. We postulate that a protein encoded on chromosome 21 may be related to the pathogenesis of moyamoya disease. Although the neuronal substrate is abnormal in Down syndrome patients, recovery from hemiplegic stroke in patients with MM-DS is comparable to recovery in patients with primary moyamoya. DOI: 10.1161/01.str.27.11.2131 PMID: 8898827 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/19582169
1. PLoS One. 2009 Jul 2;4(7):e6133. doi: 10.1371/journal.pone.0006133. Multiple horizontal gene transfer events and domain fusions have created novel regulatory and metabolic networks in the oomycete genome. Morris PF(1), Schlosser LR, Onasch KD, Wittenschlaeger T, Austin R, Provart N. Author information: (1)Department of Biological Sciences, Bowling Green State University, Bowling Green, OH, USA. pmorris@bgsu.edu Complex enzymes with multiple catalytic activities are hypothesized to have evolved from more primitive precursors. Global analysis of the Phytophthora sojae genome using conservative criteria for evaluation of complex proteins identified 273 novel multifunctional proteins that were also conserved in P. ramorum. Each of these proteins contains combinations of protein motifs that are not present in bacterial, plant, animal, or fungal genomes. A subset of these proteins were also identified in the two diatom genomes, but the majority of these proteins have formed after the split between diatoms and oomycetes. Documentation of multiple cases of domain fusions that are common to both oomycetes and diatom genomes lends additional support for the hypothesis that oomycetes and diatoms are monophyletic. Bifunctional proteins that catalyze two steps in a metabolic pathway can be used to infer the interaction of orthologous proteins that exist as separate entities in other genomes. We postulated that the novel multifunctional proteins of oomycetes could function as potential Rosetta Stones to identify interacting proteins of conserved metabolic and regulatory networks in other eukaryotic genomes. However ortholog analysis of each domain within our set of 273 multifunctional proteins against 39 sequenced bacterial and eukaryotic genomes, identified only 18 candidate Rosetta Stone proteins. Thus the majority of multifunctional proteins are not Rosetta Stones, but they may nonetheless be useful in identifying novel metabolic and regulatory networks in oomycetes. Phylogenetic analysis of all the enzymes in three pathways with one or more novel multifunctional proteins was conducted to determine the probable origins of individual enzymes. These analyses revealed multiple examples of horizontal transfer from both bacterial genomes and the photosynthetic endosymbiont in the ancestral genome of Stramenopiles. The complexity of the phylogenetic origins of these metabolic pathways and the paucity of Rosetta Stones relative to the total number of multifunctional proteins suggests that the proteome of oomycetes has few features in common with other Kingdoms. DOI: 10.1371/journal.pone.0006133 PMCID: PMC2705460 PMID: 19582169 [Indexed for MEDLINE] Conflict of interest statement: Competing Interests: The authors have declared that no competing interests exist.
http://www.ncbi.nlm.nih.gov/pubmed/23181572
1. Curr Pharm Des. 2013;19(22):4025-38. doi: 10.2174/1381612811319220012. From bortezomib to other inhibitors of the proteasome and beyond. Buac D(1), Shen M, Schmitt S, Kona FR, Deshmukh R, Zhang Z, Neslund-Dudas C, Mitra B, Dou QP. Author information: (1)Department of Oncology and Barbara Ann Karmanos Cancer Institute, Wayne State University School of Medicine, 4100 John R Street Hudson Webber Cancer Research Center Room 540.1, Detroit Michigan 48201 USA. doup@karmanos.org. The cancer drug discovery field has placed much emphasis on the identification of novel and cancer-specific molecular targets. A rich source of such targets for the design of novel anti-tumor agents is the ubiqutin-proteasome system (UP-S), a tightly regulated, highly specific pathway responsible for the vast majority of protein turnover within the cell. Because of its critical role in almost all cell processes that ensure normal cellular function, its inhibition at one point in time was deemed non-specific and therefore not worth further investigation as a molecular drug target. However, today the proteasome is one of the most promising anti-cancer drug targets of the century. The discovery that tumor cells are in fact more sensitive to proteasome inhibitors than normal cells indeed paved the way for the design of its inhibitors. Such efforts have led to bortezomib, the first FDA approved proteasome inhibitor now used as a frontline treatment for newly diagnosed multiple myeloma (MM), relapsed/refractory MM and mantle cell lymphoma. Though successful in improving clinical outcomes for patients with hematological malignancies, relapse often occurs in those who initially responded to bortezomib. Therefore, the acquisition of bortezomib resistance is a major issue with its therapy. Furthermore, some neuro-toxicities have been associated with bortezomib treatment and its efficacy in solid tumors is lacking. These observations have encouraged researchers to pursue the next generation of proteasome inhibitors, which would ideally overcome bortezomib resistance, have reduced toxicities and a broader range of anti-cancer activity. This review summarizes the success and limitations of bortezomib, and describes recent advances in the field, including, and most notably, the most recent FDA approval of carfilzomib in July, 2012, a second generation proteasome inhibitor. Other proteasome inhibitors currently in clinical trials and those that are currently experimental grade will also be discussed. DOI: 10.2174/1381612811319220012 PMCID: PMC3657018 PMID: 23181572 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/18558051
1. Actas Dermosifiliogr. 2008 Jul-Aug;99(6):431-40. [Burning mouth syndrome]. [Article in Spanish] Brufau-Redondo C(1), Martín-Brufau R, Corbalán-Velez R, de Concepción-Salesa A. Author information: (1)Servicio de Dermatología, Hospital General Universitario Reina Sofía, Murcia, Spain. Cbrufau@terra.es Burning mouth syndrome is characterized by a painful burning or stinging sensation affecting the tongue or other areas of the mouth without obvious signs of an organic cause on physical examination. A burning mouth sensation can occur in several cutaneous or systemic diseases that must be ruled out prior to making a diagnosis of burning mouth syndrome, since this term is used exclusively to refer to idiopathic forms and is included within the cutaneous sensory disorders. In most cases, patients with burning mouth syndrome have accompanying psychologic or psychiatric conditions. Consequently, the syndrome has traditionally been included among the psychogenic dermatoses. However, it is currently unclear whether psychologic factors are a cause or a consequence of the syndrome, or whether each exacerbates the other. Recent studies propose the etiology to be neurologic, either neuropathic or related to taste. PMID: 18558051 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/20635402
1. Am J Med Genet A. 2010 Aug;152A(8):2085-9. doi: 10.1002/ajmg.a.33539. Aortic dissection and moyamoya disease in Turner syndrome. Jagannath AD(1), Rastogi U, Spooner AE, Lin AE, Agnihotri AK. Author information: (1)Division of Cardiac Surgery, Massachusetts General Hospital, Boston, Massachusetts, USA. Aortic dilation and dissection are well-recognized cardiac abnormalities in women with Turner syndrome (TS), although the underlying pathophysiology is not fully understood. We report on a 46-year-old Hispanic woman who was previously diagnosed with moyamoya disease on magnetic resonance imaging after a presentation with stroke-like symptoms. Her features were consistent with TS and chromosome analysis revealed mosaicism in which 17% of the cells showed a pseudoisodicentric Y chromosome: 45,X (25)/46,X psu idic (Y)(11.2) (5). A preceding screening transthoracic echocardiogram had shown a bicuspid aortic valve (BAV) with an aortic diameter of 3.2 cm; at the time of moyamoya diagnosis, the aorta was 3.5 cm with mild aortic stenosis and mild aortic regurgitation. Four years later, the patient had had an acute aortic dissection, Stanford type A, which was repaired successfully. This case report is the third individual with TS associated with moyamoya disease and the first associated with dissection. The small number of cases does not allow detailed analysis other than noting patient age (two older than 40 years), karyotype (two others associated with isochrome Xq), and associated cardiac risk factors (one with BAV). Although this may be a chance occurrence, we hypothesize that moyamoya disease could be a manifestation of the vasculopathy in TS. DOI: 10.1002/ajmg.a.33539 PMID: 20635402 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/24694531
1. J Am Coll Cardiol. 2014 Jun 17;63(23):2541-2548. doi: 10.1016/j.jacc.2014.03.019. Epub 2014 Mar 30. Anti-PCSK9 antibody effectively lowers cholesterol in patients with statin intolerance: the GAUSS-2 randomized, placebo-controlled phase 3 clinical trial of evolocumab. Stroes E(1), Colquhoun D(2), Sullivan D(3), Civeira F(4), Rosenson RS(5), Watts GF(6), Bruckert E(7), Cho L(8), Dent R(9), Knusel B(9), Xue A(9), Scott R(9), Wasserman SM(9), Rocco M(10); GAUSS-2 Investigators. Author information: (1)Department of Vascular Medicine, Academic Medical Center, Amsterdam, the Netherlands. Electronic address: e.s.stroes@amc.uva.nl. (2)Wesley Medical Centre, Auchenflower, Australia. (3)Department of Clinical Biochemistry, Royal Prince Alfred Hospital, Camperdown, Australia. (4)Hospital Universitario Miguel Servet, Zaragoza, Spain. (5)Cardiometabolic Disorders Department, Icahn School of Medicine at Mount Sinai, New York, New York. (6)Lipid Disorders Clinic, Royal Perth Hospital, School of Medicine and Pharmacology, University of Western Australia, Perth, Australia. (7)Hopital Pitie-Salpetriere, Paris, France. (8)Preventive Cardiology and Rehabilitation, Cleveland Clinic, Cleveland, Ohio. (9)Amgen, Thousand Oaks, California. (10)Cardiovascular Medicine Department, Cleveland Clinic, Cleveland, Ohio. OBJECTIVES: This study sought to evaluate the efficacy and safety of subcutaneous evolocumab compared with oral ezetimibe in hypercholesterolemic patients who are unable to tolerate effective statin doses. BACKGROUND: Statin intolerance, which is predominantly due to muscle-related side effects, is reported in up to 10% to 20% of patients. Evolocumab, a fully human monoclonal antibody to proprotein convertase subtilisin/kexin type 9 (PCSK9), demonstrated marked reductions in plasma low-density lipoprotein cholesterol (LDL-C) in a phase 2 study in statin-intolerant patients. METHODS: The GAUSS-2 (Goal Achievement after Utilizing an Anti-PCSK9 Antibody in Statin Intolerant Subjects) trial was a 12-week, double-blind study of randomized patients (2:2:1:1) to evolocumab 140 mg every two weeks (Q2W) or evolocumab 420 mg once monthly (QM) both with daily oral placebo or subcutaneous placebo Q2W or QM both with daily oral ezetimibe 10 mg. Co-primary endpoints were percent change from baseline in LDL-C at the mean of weeks 10 and 12, and at week 12. RESULTS: Three hundred seven patients (age 62 ± 10 years; LDL-C 193 ± 59 mg/dl) were randomized. Evolocumab reduced LDL-C from baseline by 53% to 56%, corresponding to treatment differences versus ezetimibe of 37% to 39% (p <0.001). Muscle adverse events occurred in 12% of evolocumab-treated patients and 23% of ezetimibe-treated patients. Treatment-emergent adverse events and laboratory abnormalities were comparable across treatment groups. CONCLUSIONS: Robust efficacy combined with favorable tolerability makes evolocumab a promising therapy for addressing the largely unmet clinical need in high-risk patients with elevated cholesterol who are statin intolerant. (Goal Achievement After Utilizing an Anti-PCSK9 Antibody in Statin Intolerant Subjects-2; NCT01763905). Copyright © 2014 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved. DOI: 10.1016/j.jacc.2014.03.019 PMID: 24694531 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/21867529
1. Respir Res. 2011 Aug 25;12(1):115. doi: 10.1186/1465-9921-12-115. Altered surfactant homeostasis and recurrent respiratory failure secondary to TTF-1 nuclear targeting defect. Peca D(1), Petrini S, Tzialla C, Boldrini R, Morini F, Stronati M, Carnielli VP, Cogo PE, Danhaive O. Author information: (1)Department of Medical and Surgical Neonatology, Bambino Gesù Children's Hospital IRCCS, Rome, Italy. BACKGROUND: Mutations of genes affecting surfactant homeostasis, such as SFTPB, SFTPC and ABCA3, lead to diffuse lung disease in neonates and children. Haploinsufficiency of NKX2.1, the gene encoding the thyroid transcription factor-1 (TTF-1)--critical for lung, thyroid and central nervous system morphogenesis and function--causes a rare form of progressive respiratory failure designated brain-lung-thyroid syndrome. Molecular mechanisms involved in this syndrome are heterogeneous and poorly explored. We report a novel TTF-1 molecular defect causing recurrent respiratory failure episodes in an infant. METHODS: The subject was an infant with severe neonatal respiratory distress syndrome followed by recurrent respiratory failure episodes, hypopituitarism and neurological abnormalities. Lung histology and ultrastructure were assessed by surgical biopsy. Surfactant-related genes were studied by direct genomic DNA sequencing and array chromatine genomic hybridization (aCGH). Surfactant protein expression in lung tissue was analyzed by confocal immunofluorescence microscopy. For kinetics studies, surfactant protein B and disaturated phosphatidylcholine (DSPC) were isolated from serial tracheal aspirates after intravenous administration of stable isotope-labeled (2)H(2)O and (13)C-leucine; fractional synthetic rate was derived from gas chromatography/mass spectrometry (2)H and (13)C enrichment curves. Six intubated infants with no primary lung disease were used as controls. RESULTS: Lung biopsy showed desquamative interstitial pneumonitis and lamellar body abnormalities suggestive of genetic surfactant deficiency. Genetic studies identified a heterozygous ABCA3 mutation, L941P, previously unreported. No SFTPB, SFTPC or NKX2.1 mutations or deletions were found. However, immunofluorescence studies showed TTF-1 prevalently expressed in type II cell cytoplasm instead of nucleus, indicating defective nuclear targeting. This pattern has not been reported in human and was not found in two healthy controls and in five ABCA3 mutation carriers. Kinetic studies demonstrated a marked reduction of SP-B synthesis (43.2 vs. 76.5 ± 24.8%/day); conversely, DSPC synthesis was higher (12.4 vs. 6.3 ± 0.5%/day) compared to controls, although there was a marked reduction of DSPC content in tracheal aspirates (29.8 vs. 56.1 ± 12.4% of total phospholipid content). CONCLUSION: Defective TTF-1 signaling may result in profound surfactant homeostasis disruption and neonatal/pediatric diffuse lung disease. Heterozygous ABCA3 missense mutations may act as disease modifiers in other genetic surfactant defects. DOI: 10.1186/1465-9921-12-115 PMCID: PMC3179724 PMID: 21867529 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/21551164
1. Eur J Endocrinol. 2011 Jul;165(1):167-70. doi: 10.1530/EJE-11-0101. Epub 2011 May 6. Life-threatening metabolic alkalosis in Pendred syndrome. Kandasamy N(1), Fugazzola L, Evans M, Chatterjee K, Karet F. Author information: (1)Institute of Metabolic Science, University of Cambridge, Cambridge, UK. INTRODUCTION: Pendred syndrome, a combination of sensorineural deafness, impaired organification of iodide in the thyroid and goitre, results from biallelic defects in pendrin (encoded by SLC26A4), which transports chloride and iodide in the inner ear and thyroid respectively. Recently, pendrin has also been identified in the kidneys, where it is found in the apical plasma membrane of non-α-type intercalated cells of the cortical collecting duct. Here, it functions as a chloride-bicarbonate exchanger, capable of secreting bicarbonate into the urine. Despite this function, patients with Pendred syndrome have not been reported to develop any significant acid-base disturbances, except a single previous reported case of metabolic alkalosis in the context of Pendred syndrome in a child started on a diuretic. CASE REPORT: We describe a 46-year-old female with sensorineural deafness and hypothyroidism, who presented with severe hypokalaemic metabolic alkalosis during inter-current illnesses on two occasions, and who was found to be homozygous for a loss-of-function mutation (V138F) in SLC26A4. Her acid-base status and electrolytes were unremarkable when she was well. CONCLUSION: This case illustrates that, although pendrin is not usually required to maintain acid-base homeostasis under ambient condition, loss of renal bicarbonate excretion by pendrin during a metabolic alkalotic challenge may contribute to life-threatening acid-base disturbances in patients with Pendred syndrome. DOI: 10.1530/EJE-11-0101 PMCID: PMC3118492 PMID: 21551164 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/19648386
1. Mayo Clin Proc. 2009 Aug;84(8):694-701. doi: 10.4065/84.8.694. Validity of the FOUR score coma scale in the medical intensive care unit. Iyer VN(1), Mandrekar JN, Danielson RD, Zubkov AY, Elmer JL, Wijdicks EF. Author information: (1)Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN 55905, USA. OBJECTIVE: To evaluate the validity of the FOUR (Full Outline of UnResponsiveness) score (ranging from 0 to 16), a new coma scale consisting of 4 components (eye response, motor response, brainstem reflexes, and respiration pattern), when used by the staff members of a medical intensive care unit (ICU). PATIENTS AND METHODS: This interobserver agreement study prospectively evaluated the use of the FOUR score to describe the condition of 100 critically ill patients from May 1, 2007, to April 30, 2008. We compared the FOUR score to the Glasgow Coma Scale (GCS) score. For each patient, the FOUR score and the GCS score were determined by a randomly selected staff pair (nurse/fellow, nurse/consultant, fellow/fellow, or fellow/consultant). Pair wise weighted kappa values were calculated for both scores for each observer pair. RESULTS: The interrater agreement with the FOUR score was excellent (weighted kappa: eye response, 0.96; motor response, 0.97; brainstem reflex, 0.98; respiration pattern, 1.00) and similar to that obtained with the GCS (weighted kappa: eye response, 0.96; motor response, 0.97; verbal response, 0.98). In terms of the predictive power for poor neurologic outcome (Modified Rankin Scale score, 3-6), the area under the receiver operating characteristic curve was 0.75 for the FOUR score and 0.76 for the GCS score. The mortality rate for patients with the lowest FOUR score of 0 (89%) was higher than that for patients with the lowest GCS score of 3 (71%). CONCLUSION: The interrater agreement of FOUR score results was excellent among medical intensivists. In contrast to the GCS, all components of the FOUR score can be rated even when patients have undergone intubation. The FOUR score is a good predictor of the prognosis of critically ill patients and has important advantages over the GCS in the ICU setting. DOI: 10.4065/84.8.694 PMCID: PMC2719522 PMID: 19648386 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/23822094
1. Anat Histol Embryol. 2014 Jun;43(3):239-44. doi: 10.1111/ahe.12074. Epub 2013 Jul 4. 3D printing of rat salivary glands: The submandibular-sublingual complex. Cecchini MP(1), Parnigotto M, Merigo F, Marzola P, Daducci A, Tambalo S, Boschi F, Colombo L, Sbarbati A. Author information: (1)Department of Neurological, Neuropsychological, Morphological and Movement Sciences, Anatomy and Histology Section, University of Verona, Strada Le Grazie 8, 37134, Verona, Italy. The morphology and the functionality of the murid glandular complex, composed of the submandibular and sublingual salivary glands (SSC), were the object of several studies conducted mainly using magnetic resonance imaging (MRI). Using a 4.7 T scanner and a manganese-based contrast agent, we improved the signal-to-noise ratio of the SSC relating to the surrounding anatomical structures allowing to obtain high-contrast 3D images of the SSC. In the last few years, the large development in resin melting techniques opened the way for printing 3D objects starting from a 3D stack of images. Here, we demonstrate the feasibility of the 3D printing technique of soft tissues such as the SSC in the rat with the aim to improve the visualization of the organs. This approach is useful to preserve the real in vivo morphology of the SCC in living animals avoiding the anatomical shape changes due to the lack of relationships with the surrounding organs in case of extraction. It is also harmless, repeatable and can be applied to explore volumetric changes occurring during body growth, excretory duct obstruction, tumorigenesis and regeneration processes. 3D printing allows to obtain a solid object with the same shape of the organ of interest, which can be observed, freely rotated and manipulated. To increase the visibility of the details, it is possible to print the organs with a selected zoom factor, useful as in case of tiny organs in small mammalia. An immediate application of this technique is represented by educational classes. © 2013 Blackwell Verlag GmbH. DOI: 10.1111/ahe.12074 PMID: 23822094 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/21995290
1. BMC Med Genet. 2011 Oct 13;12:138. doi: 10.1186/1471-2350-12-138. Comprehensive analysis of RET common and rare variants in a series of Spanish Hirschsprung patients confirms a synergistic effect of both kinds of events. Núñez-Torres R(1), Fernández RM, Acosta MJ, Enguix-Riego Mdel V, Marbá M, Carlos de Agustín J, Castaño L, Antiñolo G, Borrego S. Author information: (1)Unidad de Gestión Clínica de Genética, Reproducción y Medicina Fetal, Instituto de Biomedicina de Sevilla, Hospital Universitario Virgen del Rocío/CSIC/Universidad de Sevilla, Sevilla, Spain. BACKGROUND: RET is the major gene associated to Hirschsprung disease (HSCR) with differential contributions of its rare and common, coding and noncoding mutations to the multifactorial nature of this pathology. In the present study, we have performed a comprehensive study of our HSCR series evaluating the involvement of both RET rare variants (RVs) and common variants (CVs) in the context of the disease. METHODS: RET mutational screening was performed by dHPLC and direct sequencing for the identification of RVs. In addition Taqman technology was applied for the genotyping of 3 RET CVs previously associated to HSCR, including a variant lying in an enhancer domain within RET intron 1 (rs2435357). Statistical analyses were performed using the SPSS v.17.0 to analyze the distribution of the variants. RESULTS: Our results confirm the strongest association to HSCR for the "enhancer" variant, and demonstrate a significantly higher impact of it in male versus female patients. Integration of the RET RVs and CVs analysis showed that in 91.66% of cases with both kinds of mutational events, the enhancer allele is in trans with the allele bearing the RET RV. CONCLUSIONS: A gender effect exists on both the transmission and distribution of rare coding and common HSCR causing mutations. In addition, these RET CVs and RVs seem to act in a synergistic way leading to HSCR phenotype. DOI: 10.1186/1471-2350-12-138 PMCID: PMC3210088 PMID: 21995290 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/15254679
1. Oncol Rep. 2004 Aug;12(2):207-11. Ectopic expression of DICE1 suppresses tumor cell growth. Wieland I(1), Sell C, Weidle UH, Wieacker P. Author information: (1)Institute of Human Genetics, Otto-von-Guericke-Universität, Magdeburg, Germany. ilse.wieland@medizin.uni-magdeburg.de The tumor suppressor gene DICE1 is located within a previously reported critical region of loss of heterozygosity on chromosome 13q14.3. Expression of the remaining DICE1 allele is down-regulated in non-small cell lung carcinomas. Ectopic expression of DICE1 cDNA by DICE1-green fluorescent protein fusion constructs resulted in inhibition of colony formation of human non-small cell lung carcinoma cell line SK-MES-1 and NCI-H520 and prostate carcinoma cell line DU145. In IGF-IR transformed Balb/c 3T3, DICE1 substantially sup-pressed growth in soft agar. These results demonstrate that DICE1 has a growth-suppressing activity and interferes with anchorage-independent growth of IGF-IR transformed tumor cells dependent upon IGF-I signaling. PMID: 15254679 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/16549820
1. J Clin Oncol. 2006 Apr 10;24(11):1672-8. doi: 10.1200/JCO.2005.04.4339. Epub 2006 Mar 20. Association of the PDCD5 locus with lung cancer risk and prognosis in smokers. Spinola M(1), Meyer P, Kammerer S, Falvella FS, Boettger MB, Hoyal CR, Pignatiello C, Fischer R, Roth RB, Pastorino U, Haeussinger K, Nelson MR, Dierkesmann R, Dragani TA, Braun A. Author information: (1)Department of Experimental Oncology and Laboratories, Thoracic Surgery, Istituto Nazionale Tumori, Milan, Italy. Comment in J Clin Oncol. 2006 Apr 10;24(11):1651-2. doi: 10.1200/JCO.2005.05.4114. PURPOSE: Whole-genome scan association analysis was carried out to identify genetic variants predictive of lung cancer risk in smokers and to confirm the identified variants in an independent sample. PATIENTS AND METHODS: A case-control study was performed using two pools consisting of DNA from 322 German smoking lung cancer patients and 273 healthy smoking controls, respectively. A replication study was carried out using 254 Italian lung adenocarcinoma (ADCA) patients and 235 healthy controls. RESULTS: Patients with genotypes GG or CG for the rs1862214 single nucleotide polymorphism, 5' upstream of the programmed cell death 5 (PDCD5) gene, compared with those with the common genotype CC showed an increased risk of lung cancer (odds ratio, 1.6; 95% CI, 1.2 to 2.1) and a higher incidence of poor clinical stage disease (hazard ratio [HR], 1.9; 95% CI, 1.1 to 3.4; P = .023), nodal involvement (HR, 1.9; 95% CI, 1.1 to 3.6; P = .033), and short-term survivorship (HR, 1.8; 95% CI, 1.2 to 2.6, P = .003). PDCD5 mRNA expression levels were approximately 2.4-fold lower in lung ADCA as compared to normal lung tissue. Human NCI-H520 cancer cells transfected with PDCD5 cDNA showed decreased colony-forming ability. CONCLUSION: These results suggest that the rs1862214 polymorphism in PDCD5 is predictive for lung cancer risk and prognosis, and that PDCD5 may represent a novel tumor suppressor gene influencing lung cancer. DOI: 10.1200/JCO.2005.04.4339 PMID: 16549820 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/18427804
1. Appl Microbiol Biotechnol. 2008 Jun;79(3):339-54. doi: 10.1007/s00253-008-1458-6. Epub 2008 Apr 22. The yeast Kluyveromyces marxianus and its biotechnological potential. Fonseca GG(1), Heinzle E, Wittmann C, Gombert AK. Author information: (1)Department of Chemical Engineering, University of São Paulo, São Paulo, São Paulo, Brazil. Strains belonging to the yeast species Kluyveromyces marxianus have been isolated from a great variety of habitats, which results in a high metabolic diversity and a substantial degree of intraspecific polymorphism. As a consequence, several different biotechnological applications have been investigated with this yeast: production of enzymes (beta-galactosidase, beta-glucosidase, inulinase, and polygalacturonases, among others), of single-cell protein, of aroma compounds, and of ethanol (including high-temperature and simultaneous saccharification-fermentation processes); reduction of lactose content in food products; production of bioingredients from cheese-whey; bioremediation; as an anticholesterolemic agent; and as a host for heterologous protein production. Compared to its congener and model organism, Kluyveromyces lactis, the accumulated knowledge on K. marxianus is much smaller and spread over a number of different strains. Although there is no publicly available genome sequence for this species, 20% of the CBS 712 strain genome was randomly sequenced (Llorente et al. in FEBS Lett 487:71-75, 2000). In spite of these facts, K. marxianus can envisage a great biotechnological future because of some of its qualities, such as a broad substrate spectrum, thermotolerance, high growth rates, and less tendency to ferment when exposed to sugar excess, when compared to K. lactis. To increase our knowledge on the biology of this species and to enable the potential applications to be converted into industrial practice, a more systematic approach, including the careful choice of (a) reference strain(s) by the scientific community, would certainly be of great value. DOI: 10.1007/s00253-008-1458-6 PMID: 18427804 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/21903771
1. Clin Cancer Res. 2011 Nov 1;17(21):6812-21. doi: 10.1158/1078-0432.CCR-11-0396. Epub 2011 Sep 8. BCR-ABL transcript dynamics support the hypothesis that leukemic stem cells are reduced during imatinib treatment. Stein AM(1), Bottino D, Modur V, Branford S, Kaeda J, Goldman JM, Hughes TP, Radich JP, Hochhaus A. Author information: (1)Oncology, Novartis Institutes for BioMedical Research, Inc., Cambridge, Massachusetts 02139, USA. andrew.stein@novartis.com Comment in Clin Cancer Res. 2011 Nov 1;17(21):6605-7. doi: 10.1158/1078-0432.CCR-11-2240. PURPOSE: Imatinib induces a durable response in most patients with Philadelphia chromosome-positive chronic myeloid leukemia, but it is currently unclear whether imatinib reduces the leukemic stem cell (LSC) burden, which may be an important step toward enabling safe discontinuation of therapy. In this article, we use mathematical models of BCR-ABL levels to make inferences on the dynamics of LSCs. EXPERIMENTAL DESIGN: Patients with at least 1 BCR-ABL transcript measurement on imatinib were included (N = 477). Maximum likelihood methods were used to test 3 potential hypotheses of the dynamics of BCR-ABL transcripts on imatinib therapy: (i) monoexponential, in which there is little, if any, decline in BCR-ABL transcripts; (ii) biexponential, in which patients have a rapid initial decrease in BCR-ABL transcripts followed by a more gradual response; and (iii) triexponential, in which patients first exhibit a biphasic decline but then have a third phase when BCR-ABL transcripts increase rapidly. RESULTS: We found that most patients treated with imatinib exhibit a biphasic decrease in BCR-ABL transcript levels, with a rapid decrease during the first few months of treatment, followed by a more gradual decrease that often continues over many years. CONCLUSIONS: We show that the only hypothesis consistent with current data on progenitor cell turnover and with the long-term, gradual decrease in the BCR-ABL levels seen in most patients is that these patients exhibit a continual, gradual reduction of the LSCs. This observation may explain the ability to discontinue imatinib therapy without relapse in some cases. ©2011 AACR DOI: 10.1158/1078-0432.CCR-11-0396 PMID: 21903771 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/11685733
1. Semin Oncol. 2001 Aug;28(4 Suppl 15):71-6. doi: 10.1016/s0093-7754(01)90159-9. Docetaxel, estramustine, plus trastuzumab in patients with metastatic androgen-independent prostate cancer. Small EJ(1), Bok R, Reese DM, Sudilovsky D, Frohlich M. Author information: (1)Urologic Oncology Program, University of California, San Francisco Comprehensive Cancer Center, San Francisco, CA 94143-1711, USA. The incidence of human epidermal growth factor receptor 2 (HER2) protein overexpression and its prognostic value are not well characterized in patients with prostate cancer. A phase I study was designed to evaluate docetaxel/estramustine plus trastuzumab, a humanized monoclonal antibody that binds to the HER2 receptor, in patients with metastatic androgen-independent prostate cancer (AIPC). HER2 positivity was not required because safety was the primary endpoint. Patients received oral estramustine 280 mg three times daily (days 1 to 5); docetaxel, 70 mg/m(2) intravenously (day 2); and trastuzumab, 2 mg/kg intravenously (days 2, 9, and 19), every 21 days until the disease progressed or toxicity became unacceptable. This regimen was well tolerated among the first 13 treated patients. Grade 4 neutropenia was seen in 10% of administered cycles. There were two episodes of febrile neutropenia and two thrombembolic events. Of the 13 patients evaluable for prostate-specific antigen (PSA) response, nine (69%) experienced a decrease in PSA level of >50%. Two (33%) of six patients with measurable disease had objective responses, and one complete response was seen on bone scan. Docetaxel/estramustine/trastuzumab appears to be a safe combination when used in the treatment of metastatic AIPC. The response data are too preliminary for speculation about the relative benefits of this 3-drug regimen compared with the combination of only docetaxel and estramustine in this clinical setting. Copyright 2001 by W.B. Saunders Company. DOI: 10.1016/s0093-7754(01)90159-9 PMID: 11685733 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/21878041
1. Expert Rev Cardiovasc Ther. 2011 Aug;9(8):959-73. doi: 10.1586/erc.11.99. Selective and specific I(f) inhibition with ivabradine: new perspectives for the treatment of cardiovascular disease. Ferrari R(1), Ceconi C. Author information: (1)Department of Cardiology, University of Ferrara, Salvatore Maugeri Foundation, IRCCS, Lumezzane, Italy. fri@unife.it Heart rate is a major determinant of myocardial oxygen demand and supply, and increased heart rate adversely affects the pathophysiology of myocardial ischemia. High resting heart rate is a risk factor in cardiovascular disease. The development of the heart rate-lowering agent ivabradine showed that heart rate was also an important treatment target, notably in coronary artery disease and heart failure. Indeed, heart rate reduction with ivabradine, a selective and specific I(f) inhibitor, reduces myocardial oxygen demand, increases diastolic perfusion time and improves energetics in ischemic myocardium. Ivabradine protects the myocardium during ischemia, improves left ventricular function in heart failure and reduces remodeling following myocardial infarction. It improves prognosis in patients with coronary artery disease, left ventricular dysfunction and heart rate ≥70 beats per minute, as well as in patients with heart failure and left ventricular dysfunction. Ivabradine is safe, well tolerated and can be used in combination with the main drugs for cardiovascular disease. DOI: 10.1586/erc.11.99 PMID: 21878041 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/23380391
1. Clin Nephrol. 2014 Aug;82(2):144-8. doi: 10.5414/CN107796. Adult hemolytic uremic syndrome associated with Streptococcus pneumoniae. Allen JC, McCulloch T, Kolhe NV. Erratum in Clin Nephrol. 2015 Feb;83(2):130. Kolh, Nitin V [corrected to Kolhe, Nitin V]. Hemolyitic uremic syndrome (HUS), characterized by triad of acute kidney injury, thrombocytopenia, and hemolytic anemia, has considerable morbidity and mortality and is known to be associated with diarrheal illness. It usually occurs after a diarrheal illness due to Shiga-toxin-producing Escherichia coli. Streptococcus pneumoniae is a rare but well recognized trigger for non-diarrhea associated HUS in children, but has not been reported in adults. We report a case of an adult presenting with pneumococcal pneumonia complicated by HUS and required renal replacement therapy. DOI: 10.5414/CN107796 PMID: 23380391 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/23498937
1. Cell. 2013 Mar 14;152(6):1285-97. doi: 10.1016/j.cell.2013.02.029. CTCF and cohesin: linking gene regulatory elements with their targets. Merkenschlager M(1), Odom DT. Author information: (1)Lymphocyte Development Group, MRC Clinical Sciences Centre, Imperial College London, Du Cane Road, London W12 0NN, UK. matthias.merkenschlager@csc.mrc.ac.uk Current epigenomics approaches have facilitated the genome-wide identification of regulatory elements based on chromatin features and transcriptional regulator binding and have begun to map long-range interactions between regulatory elements and their targets. Here, we focus on the emerging roles of CTCF and the cohesin in coordinating long-range interactions between regulatory elements. We discuss how species-specific transposable elements may influence such interactions by remodeling the CTCF binding repertoire and suggest that cohesin's association with enhancers, promoters, and sites defined by CTCF binding has the potential to form developmentally regulated networks of long-range interactions that reflect and promote cell-type-specific transcriptional programs. Copyright © 2013 Elsevier Inc. All rights reserved. DOI: 10.1016/j.cell.2013.02.029 PMID: 23498937 [Indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/pubmed/21170699
1. Wien Med Wochenschr. 2010 Dec;160(21-22):586-9. doi: 10.1007/s10354-010-0840-z. [Austrian guidance for the pharmacologic treatment of osteoporosis in postmenopausal women: Addendum 2010]. [Article in German] Dimai HP(1), Pietschmann P, Resch H, Preisinger E, Fahrleitner-Pammer A, Dobnig H, Klaushofer K; Austrian Society for Bone and Mineral Research (AuSBMR). Author information: (1)Klinische Abteilung für Endokrinologie und Stoffwechsel, Universitätsklinik für Innere Medizin, Medizinische Universität Graz, Graz, Austria. hans.dimai@medunigraz.at The Austrian Society for Bone and Mineral Research routinely publishes evidence-based guidelines for the treatment of postmenopausal osteoporosis. The fully human monoclonal antibody denosumab (Prolia(®)) has been recently approved by the European Medical Agency (EMEA) and the Food and Drug Administration (FDA) for the treatment of postmenopausal osteoporosis. Denosumab has been shown to reduce vertebral, non-vertebral,and hip-fracture risk effectively. Together with alendronate, risedronate, zoledronate, ibandronate, strontium ranelate, and raloxifene, denosumab constitutes an effective option in the treatment of postmenopausal osteoporosis. DOI: 10.1007/s10354-010-0840-z PMID: 21170699 [Indexed for MEDLINE]