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    Echocardiographic Strain Analysis for the Early Detection of Myocardial Structural Abnormality and Initiation of Drug Therapy in a Mouse Model of Dilated Cardiomyopathy.
    Ultrasound Med Biol 2017 Sep 20. Epub 2017 Sep 20.
    Department of Ultrasound, Xijing Hospital, Fourth Military Medical University, Xi'an, China; Pediatric Cardiology, Drexel University College of Medicine, Philadelphia, Pennsylvania, USA. Electronic address:
    This study aimed to evaluate the role of echocardiography-based strain analysis in the early diagnosis and guidance for management of dilated cardiomyopathy (DCM). Muscular dystrophy mice (which spontaneously develop DCM) and control (C57 BL/6 J) mice were sequentially evaluated by ultrasound biomicroscopy, conventional left ventricle (LV) measurement, two-dimensional (2-D) strain analysis and myocardial histologic analysis for 12 consecutive months. Significant alternation of LV remodeling and dysfunction could be detected by conventional echocardiography after 9 mo, by strain analysis after 5 mo and by histologic analysis after 4 mo. Read More

    Severe DCM phenotype of patient harboring RBM20 mutation S635A can be modeled by patient-specific induced pluripotent stem cell-derived cardiomyocytes.
    J Mol Cell Cardiol 2017 Sep 20. Epub 2017 Sep 20.
    Department of Cardiology and Pneumology, Universitätsmedizin Göttingen, Germany; Institute of Pharmacology and Toxicology, Technische Universität Dresden, Germany. Electronic address:
    The ability to generate patient-specific induced pluripotent stem cells (iPSCs) provides a unique opportunity for modeling heart disease in vitro. In this study, we generated iPSCs from a patient with dilated cardiomyopathy (DCM) caused by a missense mutation S635A in RNA-binding motif protein 20 (RBM20) and investigated the functionality and cell biology of cardiomyocytes (CMs) derived from patient-specific iPSCs (RBM20-iPSCs). The RBM20-iPSC-CMs showed abnormal distribution of sarcomeric α-actinin and defective calcium handling compared to control-iPSC-CMs, suggesting disorganized myofilament structure and altered calcium machinery in CMs of the RBM20 patient. Read More

    Myofilament remodeling and function is more impaired in peripartum cardiomyopathy compared to dilated cardiomyopathy and ischemic heart disease.
    Am J Pathol 2017 Sep 18. Epub 2017 Sep 18.
    Department of Physiology, VU University Medical Center, Amsterdam, the Netherlands; Amsterdam Cardiovascular Sciences, Amsterdam, the Netherlands; Netherlands Heart Institute, Utrecht, the Netherlands.
    Peripartum cardiomyopathy (PPCM) and dilated cardiomyopathy (DCM) show similarities in clinical presentation. However, while DCM patients do not recover and slowly deteriorate further, PPCM patients show either a fast cardiac deterioration or complete recovery. The aim of this study was to assess if underlying cellular changes can explain the clinical similarities and differences in the two diseases. Read More

    Intercalated disc in failing hearts from patients with dilated cardiomyopathy: Its role in the depressed left ventricular function.
    PLoS One 2017 21;12(9):e0185062. Epub 2017 Sep 21.
    Cardiocirculatory Unit, Health Research Institute La Fe, Valencia, Spain.
    Alterations in myocardial structure and reduced cardiomyocyte adhesions have been previously described in dilated cardiomyopathy (DCM). We studied the transcriptome of cell adhesion molecules in these patients and their relationships with left ventricular (LV) function decay. We also visualized the intercalated disc (ID) structure and organization. Read More

    Fatal Cobalt Toxicity after a Non-Metal-on-Metal Total Hip Arthroplasty.
    Case Rep Orthop 2017 27;2017:9123684. Epub 2017 Aug 27.
    Department of Orthopedic Surgery, Medical Center Leeuwarden, Henri Dunantweg 2, 8934 AD Leeuwarden, Netherlands.
    This case illustrates the potential for systemic cobalt toxicity in non-metal-on-metal bearings and its potentially devastating consequences. We present a 71-year-old male with grinding sensations in his right hip following ceramic-on-ceramic total hip arthroplasty (THA). After diagnosing a fractured ceramic liner, the hip prosthesis was revised into a metal-on-polyethylene bearing. Read More

    Human herpesvirus 6-induced inflammatory cardiomyopathy in immunocompetent children.
    Ann Pediatr Cardiol 2017 Sep-Dec;10(3):259-268
    Departement of Pediatrics, UTSW Medical Center, Dallas, TX, USA.
    Over the last decade, human herpesvirus 6 (HHV-6) has been implicated in the etiology of pediatric myocarditis and subsequent dilated cardiomyopathy (DCM). This review provides an overview of recent literature investigating the pathophysiological relevance of HHV-6 in inflammatory cardiomyopathy. We examined 11 cases of previously published pediatric myocarditis and/or DCM associated with HHV-6 and also our experience of detection of virus particles in vascular endothelium of HHV-6 positive endomyocardial biopsy tissue by electron microscopy. Read More

    Spanish Implantable Cardioverter-defibrillator Registry. 13th Official Report of the Spanish Society of Cardiology Electrophysiology and Arrhythmias Section (2016).
    Rev Esp Cardiol (Engl Ed) 2017 Sep 8. Epub 2017 Sep 8.
    Sección de Electrofisiología y Arritmias, Sociedad Española de Cardiología, Madrid, Spain.
    Introduction And Objectives: To report the findings of the Spanish Implantable Cardioverter-defibrillator Registry for 2016 compiled by the Electrophysiology and Arrhythmias Section of the Spanish Society of Cardiology.

    Methods: Prospective data were voluntarily recorded on a data collection form and send to the Spanish Society of Cardiology by each implantation team.

    Results: Overall, 5673 device implantations were reported, representing 85% of the estimated total number of implantations. Read More

    J Zoo Wildl Med 2017 Sep;48(3):829-841
    Between 2006 and 2015, a high incidence of dilated cardiomyopathy (DCM) was diagnosed in a captive population of banded mongooses (Mungos mungo) at Chester Zoo, United Kingdom. The aim of this study was to characterize DCM in these mongooses in order to raise awareness of this condition and help inform management and clinical decisions. Prospective clinical assessments, including echocardiography, radiography, and cardiac biomarkers, were carried out in four mongooses remaining in the collection. Read More

    Sudden Death Mechanisms in Non-ischemic Cardiomyopathies; Insights gleaned from clinical implantable cardioverter-defibrillator trials.
    Heart Rhythm 2017 Sep 14. Epub 2017 Sep 14.
    Department of Cardiovascular Diseases, Mayo Clinic College of Medicine and Sciences, Rochester, MN. Electronic address:
    Sudden cardiac death (SCD) represents a major cause of death among patients with heart failure. While scar-based, macro-reentrant ventricular tachycardia and ventricular fibrillation (VT/VF) is the primary etiology for SCD among patients with ischemic cardiomyopathy, a more diverse set of mechanisms and substrates are likely at play for the diverse group of patients characterized by non-ischemic, dilated cardiomyopathy (NICM). These causes may include scar-based re-entry, but also neurohormonal stimulation (sympathetic, parasympathetic, renin-angiotensin-aldosterone), inflammation, and non-arrhythmic processes occurring in the context of a genetic predisposition. Read More

    Detecting the genetic link between Alzheimer's disease and obesity using bioinformatics analysis of GWAS data.
    Oncotarget 2017 Aug 8;8(34):55915-55919. Epub 2017 Jul 8.
    Shandong Provincial Research Center for Bioinformatic Engineering and Technique, School of Life Sciences, Shandong University of Technology, Zibo, P. R. China.
    Alzheimer's disease (AD) represents the major form of dementia in the elderly. In recent years, accumulating evidence indicate that obesity may act as a risk factor for AD, while the genetic link between the two conditions remains unclear. This bioinformatics analysis aimed to detect the genetic link between AD and obesity on single nucleotide polymorphisms (SNPs), gene, and pathway levels based on genome-wide association studies data. Read More

    [Efficiency of immunosuppressive therapy in virus-negative and virus-positive patients with morphologically verified lymphocytic myocarditis].
    Ter Arkh 2017 ;89(8):57-67
    I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia, Moscow, Russia.
    Aim: To evaluate the efficiency of immunosuppressive therapy (IST) in virus-negative (V-) and virus-positive (V+) patients with lymphocytic myocarditis (LM).

    Subjects And Methods: 60 patients (45 males) (mean age 46.7±11. Read More

    Bioinformatics method identifies potential biomarkers of dilated cardiomyopathy in a human induced pluripotent stem cell-derived cardiomyocyte model.
    Exp Ther Med 2017 Oct 28;14(4):2771-2778. Epub 2017 Jul 28.
    Department of Cardiothoracic Surgery, The Affiliated Huai'an Hospital of Xuzhou Medical University, Huai'an, Jiangsu 223002, P.R. China.
    Dilated cardiomyopathy (DCM) is the most common type of cardiomyopathy that account for the majority of heart failure cases. The present study aimed to reveal the underlying molecular mechanisms of DCM and provide potential biomarkers for detection of this condition. The public dataset of GSE35108 was downloaded, and 4 normal induced pluripotent stem cell (iPSC)-derived cardiomyocytes (N samples) and 4 DCM iPSC-derived cardiomyocytes (DCM samples) were utilized. Read More

    Clinical Yield of Familial Screening After Sudden Death in Young Subjects: The French Experience.
    Circ Arrhythm Electrophysiol 2017 Sep;10(9)
    From the l'institut du thorax, INSERM, CNRS, UNIV Nantes, CHU Nantes (P.Q., F.K., A.T., B.G., R.R., J.B., J.-J.S., V.P., J.B.G.); Service de cardiologie, CHU de Rennes, (P.M.); Service de cardiologie, CHU de Brest (J.M.); Service de cardiologie, CHU de Tours (D.B.); and Service de cardiologie, Institut Lyric, CHU de Bordeaux, France (F.S.).
    Background: After sudden cardiac death with negative autopsy, clinical screening of relatives identifies a high proportion of inherited arrhythmia syndrome. However, the efficacy of this screening in families not selected by autopsy has never been assessed. We aim to investigate the value of clinical screening in relatives of all subjects who died suddenly before 45 years of age. Read More

    Pediatric Cardiomyopathies.
    Circ Res 2017 Sep;121(7):855-873
    From the Department of Pediatrics, Columbia University Medical Center, New York, NY (T.M.L., W.K.C., L.J.A.); Department of Pediatrics, Albert Einstein College of Medicine, The Children's Hospital at Montefiore, Bronx, NY (D.T.H., J.M.L.); Department of Pediatrics, Stollery Children's Hospital, University of Alberta, Edmonton, Canada (P.K.); Department of Pediatrics, The Heart Institute, Le Bonheur Children's Hospital, Memphis, TN (J.A.T.); Indiana University School of Medicine, Indianapolis (S.M.W.); Department of Cardiology, Boston Children's Hospital, MA (S.D.C.); Department of Pediatrics, Cincinnati Children's Hospital Medical Center, OH (J.L.J., E.M.M.); Department of Pediatrics, Children's Hospital of Philadelphia, PA (J.W.R.); Department of Pediatrics, Washington University School of Medicine, St. Louis, MO (C.D.C.); Department of Pediatrics, Primary Children's Hospital, Salt Lake City, UT (A.K.L.); Department of Pediatrics, Ann and Robert H. Lurie Children's Hospital, Chicago, IL (P.T.T.); and Department of Pediatrics, Wayne State University School of Medicine and Children's Hospital of Michigan, Detroit (J.D.C., H.R., A.H., S.E.L.).
    Pediatric cardiomyopathies are rare diseases with an annual incidence of 1.1 to 1.5 per 100 000. Read More

    Inflammatory Cardiomyopathic Syndromes.
    Circ Res 2017 Sep;121(7):803-818
    From the Houston Methodist DeBakey Heart and Vascular Center (B.H.T.), TX; University of Miami Leonard Miller School of Medicine, FL (J.M.H.); and Interdisciplinary Stem Cell Institute, Miami, FL (J.M.H.).
    Inflammatory activation occurs in nearly all forms of myocardial injury. In contrast, inflammatory cardiomyopathies refer to a diverse group of disorders in which inflammation of the heart (or myocarditis) is the proximate cause of myocardial dysfunction, causing injury that can range from a fully recoverable syndrome to one that leads to chronic remodeling and dilated cardiomyopathy. The most common cause of inflammatory cardiomyopathies in developed countries is lymphocytic myocarditis most commonly caused by a viral pathogenesis. Read More

    Dilated Cardiomyopathy: Genetic Determinants and Mechanisms.
    Circ Res 2017 Sep;121(7):731-748
    From the Center for Genetic Medicine, Northwestern University Feinberg School of Medicine, Chicago IL (E.M.M.); and Cardiovascular Institute, University of Colorado Anschutz Medical Campus, Aurora (L.M.).
    Nonischemic dilated cardiomyopathy (DCM) often has a genetic pathogenesis. Because of the large number of genes and alleles attributed to DCM, comprehensive genetic testing encompasses ever-increasing gene panels. Genetic diagnosis can help predict prognosis, especially with regard to arrhythmia risk for certain subtypes. Read More

    Classification, Epidemiology, and Global Burden of Cardiomyopathies.
    Circ Res 2017 Sep;121(7):722-730
    From the Imperial College London, United Kingdom (W.J.M.); Hypertrophic Cardiomyopathy Institute, Division of Cardiology, Tufts Medical Center, Boston, MA (B.J.M.); and Department of Cardiac, Thoracic and Vascular Sciences, University of Padua Medical School, Italy (G.T.).
    In the past 25 years, major advances were achieved in the nosography of cardiomyopathies, influencing the definition and taxonomy of this important chapter of cardiovascular disease. Nearly, 50% of patients dying suddenly in childhood or adolescence or undergoing cardiac transplantation are affected by cardiomyopathies. Novel cardiomyopathies have been discovered (arrhythmogenic, restrictive, and noncompacted) and added to update the World Health Organization classification. Read More

    A retrospective observational study of patients with dilated cardiomyopathy undergoing non-cardiac surgery.
    Anaesth Intensive Care 2017 Sep;45(5):619-623
    The perioperative risks and factors associated with adverse cardiac outcomes in patients with dilated cardiomyopathy undergoing non-cardiac surgery are unknown. Interrogation of the Nelson Hospital transthoracic echocardiogram database identified 127 patients with dilated cardiomyopathy who satisfied the study criteria and underwent non-cardiac surgery between June 1999 and July 2013. Demographic and clinical data along with postoperative death within 30 days or a major adverse cardiac event were retrieved and analysed. Read More

    Homozygous EEF1A2 mutation causes dilated cardiomyopathy, failure to thrive, global developmental delay, epilepsy and early death.
    Hum Mol Genet 2017 Sep;26(18):3545-3552
    Division of Newborn Medicine.
    Eukaryotic elongation factor 1A (EEF1A), is encoded by two distinct isoforms, EEF1A1 and EEF1A2; whereas EEF1A1 is expressed almost ubiquitously, EEF1A2 expression is limited such that it is only detectable in skeletal muscle, heart, brain and spinal cord. Currently, the role of EEF1A2 in normal cardiac development and function is unclear. There have been several reports linking de novo dominant EEF1A2 mutations to neurological issues in humans. Read More

    Natural genetic variation of the cardiac transcriptome in non-diseased donors and patients with dilated cardiomyopathy.
    Genome Biol 2017 Sep 14;18(1):170. Epub 2017 Sep 14.
    Cardiovascular and Metabolic Sciences, Max-Delbrück-Center for Molecular Medicine (MDC) in the Helmholtz Association, Robert-Rössle-Str. 10, 13125, Berlin, Germany.
    Background: Genetic variation is an important determinant of RNA transcription and splicing, which in turn contributes to variation in human traits, including cardiovascular diseases.

    Results: Here we report the first in-depth survey of heart transcriptome variation using RNA-sequencing in 97 patients with dilated cardiomyopathy and 108 non-diseased controls. We reveal extensive differences of gene expression and splicing between dilated cardiomyopathy patients and controls, affecting known as well as novel dilated cardiomyopathy genes. Read More

    MEF2C loss-of-function mutation associated with familial dilated cardiomyopathy.
    Clin Chem Lab Med 2017 Sep 13. Epub 2017 Sep 13.
    Background: The MADS-box transcription factor myocyte enhancer factor 2C (MEF2C) is required for the cardiac development and postnatal adaptation and in mice-targeted disruption of the MEF2C gene results in dilated cardiomyopathy (DCM). However, in humans, the association of MEF2C variation with DCM remains to be investigated.

    Methods: The coding regions and splicing boundaries of the MEF2C gene were sequenced in 172 unrelated patients with idiopathic DCM. Read More

    Malignant extra-adrenal pelvic paraganglioma in a paediatric patient.
    Ecancermedicalscience 2017 23;11:761. Epub 2017 Aug 23.
    Anatomical Pathology Division, 'Pedro De Elizalde' Children's Hospital, Montes de Oca Avenue 40, Buenos Aires C1270AAN, Argentina.
    The extra-adrenal paraganglioma is a neoplasm originating in regional structures, uncommon in paediatrics. We report on a case of a 13-year-old patient who began with severe arterial hypertension, tachycardia, dilated cardiomyopathy and elevated levels of catecholamines in the blood and urine. The presence of a retrovesical pelvic mass in contact with the right vaginal dome was determined by imaging studies. Read More

    Serum paraoxonase activity in patients with ischaemic and nonischaemic dilated cardiomyopathy.
    Acta Cardiol 2017 Sep 12:1-6. Epub 2017 Sep 12.
    a Department of Cardiology, Faculty of Medicine , Uludag University , Bursa , Turkey.
    Background: This study examined whether the serum PON1 activity is different in patients with ischaemic dilated cardiomyopathy (IDCM) and nonischaemic dilated cardiomyopathy (NDCM) and the relation between the serum PON1 activity and serum pro-BNP levels.

    Methods And Results: In this study, we enrolled 60 patients with left ventricular systolic failure (New York Heart Association [NYHA] class III-IV) and a left ventricular ejection fraction (EF) < 40% as determined by echocardiography and 30 healthy subjects. The patients with systolic heart failure were divided into two groups: patients with IDCM and patients with NDCM. Read More

    The Use of a Novel Heart Failure Agent in the Treatment of Pregnancy-Associated Cardiomyopathy.
    Case Rep Cardiol 2017 14;2017:9561405. Epub 2017 Aug 14.
    Department of Cardiovascular Sciences, Morsani College of Medicine, University of South Florida, Tampa, FL, USA.
    Peripartum cardiomyopathy is an uncommon, pregnancy-related form of dilated cardiomyopathy that is associated with development of new-onset left ventricular dysfunction. Its etiology is presently unknown, but current standard of care involves the use of typical drug therapy for the treatment of heart failure. Pregnancy-associated cardiomyopathy (PACM) is a similar condition that refers to patients who develop such symptoms prior to the last month of pregnancy. Read More

    Circulating ANGPTL2 Levels Increase in Humans and Mice Exhibiting Cardiac Dysfunction.
    Circ J 2017 Sep 8. Epub 2017 Sep 8.
    Department of Molecular Genetics, Graduate School of Medical Sciences, Kumamoto University.
    Background: Recently, it was reported that angiopoietin-like protein 2 (ANGPTL2) secreted from a pathologically stressed heart accelerates cardiac dysfunction in an autocrine/paracrine manner, and that suppression of ANGPTL2 production in the heart restored cardiac function and myocardial energy metabolism, thereby blocking heart failure (HF) development. Interestingly, circulating ANGPTL2 concentrations reportedly increase in HF patients, suggesting a possible endocrine effect on cardiac dysfunction. However, it remains unclear why circulating ANGPTL2 increases in those subjects and whether circulating ANGPTL2 alters cardiac function in an endocrine manner. Read More

    Histamine 2 receptor antagonism elicits protection against doxorubicin-induced cardiotoxicity in rodent model.
    Mol Cell Biochem 2017 Sep 8. Epub 2017 Sep 8.
    Department of Pharmacology, Sri Vishnu College of Pharmacy, Bhimavaram, West Godavari, Andhra Pradesh, India.
    Doxorubicin (DOX), an anthracycline-based antibiotic, is regularly used in the management of carcinomas, and haematological malignancies have been downplayed in chemotherapy because of its ability to induce dilated cardiomyopathy (DCM). Dexrazoxane is approved to combat the cardiotoxicity, but limited by its adverse effects. Redox imbalance and reactive oxygen species generation plays major role in DOX-induced cardiotoxicity. Read More

    TRAF6: A player in CVB3-induced myocarditis?
    Cytokine 2017 Sep 5. Epub 2017 Sep 5.
    University Hospital Tübingen Medical Clinic, Department of Sports Medicine, Hoppe-Seyler-Str. 6, D-72076 Tübingen, Germany. Electronic address:
    Coxsackievirus B3 (CVB3) is an important inducer of myocarditis, which, in susceptible individuals, can chronify and eventually lead to the development of dilated cardiomyopathy and heart failure. The respective mechanisms are not completely understood. Here, we analyzed expression of the TRAF6 gene, encoding TNF receptor-associated factor 6 (TRAF6), a signal transduction scaffold protein that acts downstream of cytokine receptors, in heart tissue of susceptible and non-susceptible mouse strains. Read More

    MicroRNA miR-301a is a novel cardiac regulator of Cofilin-2.
    PLoS One 2017 8;12(9):e0183901. Epub 2017 Sep 8.
    Department of Internal Medicine III (Cardiology, Angiology, Intensive Care), University Medical Center Kiel, Kiel, Germany.
    Calsarcin-1 deficient mice develop dilated cardiomyopathy (DCM) phenotype in pure C57BL/6 genetic background (Cs1-ko) despite severe contractile dysfunction and robust activation of fetal gene program. Here we performed a microRNA microarray to identify the molecular causes of this cardiac phenotype that revealed the dysregulation of several microRNAs including miR-301a, which was highly downregulated in Cs1-ko mice compared to the wild-type littermates. Cofilin-2 (Cfl2) was identified as one of the potential targets of miR-301a using prediction databases, which we validated by luciferase assay and mutation of predicted binding sites. Read More

    Exogenous T3 toxicosis following consumption of a contaminated weight loss supplement.
    Endocrinol Diabetes Metab Case Rep 2017 29;2017. Epub 2017 Aug 29.
    Regional Centre for Endocrinology, Royal Victoria Hospital, BelfastUK.
    A 42-year-old male presented with a one-week history of palpitations and sweating episodes. The only significant history was of longstanding idiopathic dilated cardiomyopathy. Initial ECG demonstrated a sinus tachycardia. Read More

    Urinary Proteomics in Predicting Heart Transplantation Outcomes (uPROPHET)-Rationale and database description.
    PLoS One 2017 7;12(9):e0184443. Epub 2017 Sep 7.
    Studies Coordinating Centre, Research Unit Hypertension and Cardiovascular Epidemiology, KU Leuven Department of Cardiovascular Sciences, University of Leuven, Leuven, Belgium.
    Objectives: Urinary Proteomics in Predicting Heart Transplantation Outcomes (uPROPHET; NCT03152422) aims: (i) to construct new multidimensional urinary proteomic (UP) classifiers that after heart transplantation (HTx) help in detecting graft vasculopathy, monitoring immune system activity and graft performance, and in adjusting immunosuppression; (ii) to sequence UP peptide fragments and to identify key proteins mediating HTx-related complications; (iii) to validate UP classifiers by demonstrating analogy between UP profiles and tissue proteomic signatures (TP) in diseased explanted hearts, to be compared with normal donor hearts; (iv) and to identify new drug targets. This article describes the uPROPHET database construction, follow-up strategies and baseline characteristics of the HTx patients.

    Methods: HTx patients enrolled at the University Hospital Gasthuisberg (Leuven) collected mid-morning urine samples. Read More

    Percutaneous extracorporeal membrane oxygenation in electrical storm: five case reports addressing efficacy, transferring allowance or radiofrequency ablation support.
    Eur Heart J Acute Cardiovasc Care 2017 Sep 1:2048872617730036. Epub 2017 Sep 1.
    Department of Cardiology, University Hospital of Salamanca - IBSAL, Salamanca, Spain.
    Extracorporeal membrane oxygenation systems have undergone rapid technological improvements and are now feasible options for medium-term support of severe cardiac or pulmonary failure. We report five cases of electrical storm that was rescued by the insertion of peripheral veno-arterial extracorporeal membrane oxygenation systems. This device could help to restore systemic circulation as well as permitting organ perfusion in patients with cardiogenic shock in relation to electrical storm thus achieving greater electrical stability. Read More

    An exploratory investigation of echocardiographic parameters and the effects of posture on cardiac structure and function in the Livingstone's fruit bat (Pteropus livingstonii).
    Vet Radiol Ultrasound 2017 Sep 6. Epub 2017 Sep 6.
    Durrell Wildlife Conservation Trust, Les Augrès Manor, La Profonde Rue, Trinity, Jersey JE3 5BP, Channel Islands.
    There is growing evidence that dilated cardiomyopathy may be a major cause of death in captive Livingstone's fruit bats (Pteropus livingstonii). Therefore, the primary aim of this prospective, exploratory study was to examine whether a systematic cardiac ultrasound protocol is feasible in this critically endangered species and to report basic measures of cardiac structure and function from a cohort of apparently healthy bats. A secondary aim was to test the effect posture (dorsal recumbency vs. Read More

    Tead1 is required for maintaining adult cardiomyocyte function, and its loss results in lethal dilated cardiomyopathy.
    JCI Insight 2017 Sep 7;2(17). Epub 2017 Sep 7.
    Division of Cardiology, Department of Pediatrics, University of Texas (UT) Health McGovern Medical School, Houston, Texas, USA.
    Heart disease remains the leading cause of death worldwide, highlighting a pressing need to identify novel regulators of cardiomyocyte (CM) function that could be therapeutically targeted. The mammalian Hippo/Tead pathway is critical in embryonic cardiac development and perinatal CM proliferation. However, the requirement of Tead1, the transcriptional effector of this pathway, in the adult heart is unknown. Read More

    Knockout of the ATPase inhibitory factor 1 protects the heart from pressure overload-induced cardiac hypertrophy.
    Sci Rep 2017 Sep 5;7(1):10501. Epub 2017 Sep 5.
    Department of Nutrition Sciences, University of Alabama at Birmingham, Birmingham, Alabama, 35294, USA.
    Mitochondrial ATP synthase catalyzes the coupling of oxidative phosphorylation. Under pathological conditions, ATP synthase hydrolyzes ATP to replenish protons from the matrix into the intermembrane space, sustaining mitochondrial membrane potential. ATPase inhibitory factor 1 (IF1) is a nuclear-encoded, ATP synthase-interacting protein that selectively inhibits the hydrolysis activity of ATP synthase, which may render the protective role of IF1 in ischemic hearts. Read More

    CELF1 Mediates Connexin 43 mRNA Degradation in Dilated Cardiomyopathy.
    Circ Res 2017 Sep 5. Epub 2017 Sep 5.
    Institute of Biomedical Sciences, Academia Sinica
    Rationale: Downregulation of connexin 43 (Cx43), the major cardiac gap junction protein, is often associated with arrhythmia, dilated cardiomyopathy (DCM) and heart failure. However, the cause of the reduced expression remains elusive. Re-induction of a nuclear RNA-binding protein CUGBP, Elav-like family member 1 (CELF1) in the adult heart has been implicated in the cardiac pathogenesis of myotonic dystrophy type 1 (DM1). Read More

    Complex phenotype linked to a mutation in exon 11 of the lamin A/C gene: Hypertrophic cardiomyopathy, atrioventricular block, severe dyslipidemia and diabetes.
    Rev Port Cardiol 2017 Sep 3;36(9):669.e1-669.e4. Epub 2017 Sep 3.
    Cardiology Department, Santa Maria University Hospital, CHLN, CAML, CCUL, Faculty of Medicine, University of Lisbon, Lisbon, Portugal.
    The lamin A/C (LMNA) gene encodes lamins A and C, which have an important role in nuclear cohesion and chromatin organization. Mutations in this gene usually lead to the so-called laminopathies, the primary cardiac manifestations of which are dilated cardiomyopathy and intracardiac conduction defects. Some mutations, associated with lipodystrophy but not cardiomyopathy, have been linked to metabolic abnormalities such as diabetes and severe dyslipidemia. Read More

    Lessons from a tarantula: new insights into myosin interacting-heads motif evolution and its implications on disease.
    Biophys Rev 2017 Sep 4. Epub 2017 Sep 4.
    Centro de Biología Estructural "Humberto Fernández-Morán", Instituto Venezolano de Investigaciones Científicas (IVIC), Apdo. 20632, Caracas, 1020A, Venezuela.
    Tarantula's leg muscle thick filament is the ideal model for the study of the structure and function of skeletal muscle thick filaments. Its analysis has given rise to a series of structural and functional studies, leading, among other things, to the discovery of the myosin interacting-heads motif (IHM). Further electron microscopy (EM) studies have shown the presence of IHM in frozen-hydrated and negatively stained thick filaments of striated, cardiac, and smooth muscle of bilaterians, most showing the IHM parallel to the filament axis. Read More

    Transcriptional and epigenetic analyses of the DMD locus reveal novel cis‑acting DNA elements that govern muscle dystrophin expression.
    Biochim Biophys Acta 2017 Aug 31. Epub 2017 Aug 31.
    Department of Pharmacy and Biotechnology, University of Bologna, Bologna, Italy; CIRI Health Sciences & Technologies (HST), Bologna, Italy. Electronic address:
    The dystrophin gene (DMD) is the largest gene in the human genome, mapping on the Xp21 chromosome locus. It spans 2.2Mb and accounts for approximately 0,1% of the entire human genome. Read More

    A Dutch MYH7 founder mutation, p.(Asn1918Lys), is associated with early onset cardiomyopathy and congenital heart defects.
    Neth Heart J 2017 Sep 1. Epub 2017 Sep 1.
    Department of Clinical Genetics, Leiden University Medical Centre, Leiden, The Netherlands.
    Background: Mutations in the myosin heavy chain 7 (MYH7) gene commonly cause cardiomyopathy but are less frequently associated with congenital heart defects.

    Methods: In this study, we describe a mutation in the MYH7 gene, c. 5754C > G; p. Read More

    Posttranslational modifications of calcium/calmodulin-dependent protein kinase IIδ and its downstream signaling in human failing hearts.
    Am J Transl Res 2017 15;9(8):3573-3585. Epub 2017 Aug 15.
    Department of Pharmacology & Toxicology, Faculty of Pharmacy, Comenius UniversityBratislava, Slovak Republic.
    Background: In human failing hearts (HF) of different origin (coronary artery disease-CAD, dilated-DCM, restrictive and hypertrophic cardiomyopathy-OTHER), we investigated the active forms of Ca(2+)/calmodulin-dependent protein kinase IIδ (p-Thr(287)-CaMKIIδ, oxMet(281/282)-CaMKIIδ) and their role in phenotypes of the disease.

    Methods And Results: Although basic diagnostic and clinical markers indicating the attenuated cardiac contractility and remodeling were comparable in HF groups, CaMKIIδ-mediated axis was different. P-Thr(287)-CaMKIIδ was unaltered in CAD group, whereas it was upregulated in non-ischemic cardiomyopathic groups. Read More

    Ivabradine in Children With Dilated Cardiomyopathy and Symptomatic Chronic Heart Failure.
    J Am Coll Cardiol 2017 Sep;70(10):1262-1272
    Royal Brompton Hospital and Imperial College, London, United Kingdom.
    Background: Heart rate reduction as a therapeutic target has been investigated in adults with heart failure (HF). Ivabradine has shown promising efficacy, but has not been evaluated in children. Currently, treatment recommendations for chronic pediatric HF are based mainly on chronic HF guidelines for adults. Read More

    Impaired calcium homeostasis is associated with sudden cardiac death and arrhythmias in a genetic equivalent mouse model of the human HRC-Ser96Ala variant.
    Cardiovasc Res 2017 Sep;113(11):1403-1417
    Department of Molecular Biology, Center of Basic Research, Biomedical Research Foundation, Academy of Athens, Soranou Efessiou 4, 115 27, Athens, Greece.
    Aims: The histidine-rich calcium-binding protein (HRC) Ser96Ala variant has previously been identified as a potential biomarker for ventricular arrhythmias and sudden cardiac death in patients with idiopathic dilated cardiomyopathy. Herein, the role of this variant in cardiac pathophysiology is delineated through a novel mouse model, carrying the human mutation in the homologous mouse position.

    Methods And Results: The mouse HRC serine 81, homologous to human HRC serine 96, was mutated to alanine, using knock-in gene targeting. Read More

    Evaluation of Contamination Risks with Coxsackievirus B4 E2 in Swiss Albino Mice Stools.
    Curr Microbiol 2017 Aug 30. Epub 2017 Aug 30.
    Unité de Recherche UR17ES30 "Génomique, Biotechnologie et Stratégies Antivirales»", Institut Supérieur de Biotechnologie, Université de Monastir, BP74, Avenue Tahar Hadded, 5000, Monastir, Tunisia.
    Coxsackie B4 (CV-B4), is a major cause of viral myocarditis, dilated cardiomyopathy, and pancreatitis. Like other human enteroviruses, CV-B4 is ubiquitous, excreted in the stool, transmitted by fecal-oral route, and persists in the environment. In the context of studies on CV-B4 infection, it is important to investigate how this virus can be eliminated and to show the possibility of contamination risk with a CV-B4 E2 infected Swiss albino mice. Read More

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