cardiomyopathies

Summary

Summary: A group of diseases in which the dominant feature is the involvement of the CARDIAC MUSCLE itself. Cardiomyopathies are classified according to their predominant pathophysiological features (DILATED CARDIOMYOPATHY; HYPERTROPHIC CARDIOMYOPATHY; RESTRICTIVE CARDIOMYOPATHY) or their etiological/pathological factors (CARDIOMYOPATHY, ALCOHOLIC; ENDOCARDIAL FIBROELASTOSIS).

Top Publications

  1. ncbi Diabetic cardiomyopathy revisited
    Sihem Boudina
    Division of Endocrinology, Metabolism and Diabetes and Program in Human Molecular Biology and Genetics, University of Utah School of Medicine, Salt Lake City 84112, USA
    Circulation 115:3213-23. 2007
  2. ncbi Classification of the cardiomyopathies: a position statement from the European Society Of Cardiology Working Group on Myocardial and Pericardial Diseases
    Perry Elliott
    Hadassah University Hospital Ein Kerem, Kirjat Hadassah, Jerusalem 91120, Israel
    Eur Heart J 29:270-6. 2008
  3. ncbi Diabetic cardiomyopathy: evidence, mechanisms, and therapeutic implications
    Zhi You Fang
    University of Queensland, Brisbane, 4012, Australia
    Endocr Rev 25:543-67. 2004
  4. ncbi Cardiovascular T2-star (T2*) magnetic resonance for the early diagnosis of myocardial iron overload
    L J Anderson
    Cardiovascular MR Unit, Royal Brompton Hospital, London, UK
    Eur Heart J 22:2171-9. 2001
  5. pmc Desmin-related cardiomyopathy: an unfolding story
    Patrick M McLendon
    Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio 45229 3039, USA
    Am J Physiol Heart Circ Physiol 301:H1220-8. 2011
  6. ncbi Doxorubicin-induced cardiomyopathy from the cardiotoxic mechanisms to management
    Genzou Takemura
    Second Department of Internal Medicine, Gifu University School of Medicine, Gifu, Japan
    Prog Cardiovasc Dis 49:330-52. 2007
  7. ncbi Myocardial steatosis is an independent predictor of diastolic dysfunction in type 2 diabetes mellitus
    Luuk J Rijzewijk
    Department of Endocrinology, VU University Medical Center, Amsterdam, The Netherlands
    J Am Coll Cardiol 52:1793-9. 2008
  8. ncbi Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functio
    Barry J Maron
    Circulation 113:1807-16. 2006
  9. ncbi Cardiogenetics, neurogenetics, and pathogenetics of left ventricular hypertrabeculation/noncompaction
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Postfach 20, 1180 Vienna, Austria, Europe
    Pediatr Cardiol 30:659-81. 2009
  10. pmc Non-invasive evaluation of the myocardial substrate of cardiac amyloidosis by gadolinium cardiac magnetic resonance
    E Perugini
    Institute of Cardiology, University of Bologna, S Orsola Malpighi Hospital, Bologna, Italy
    Heart 92:343-9. 2006

Detail Information

Publications297 found, 100 shown here

  1. ncbi Diabetic cardiomyopathy revisited
    Sihem Boudina
    Division of Endocrinology, Metabolism and Diabetes and Program in Human Molecular Biology and Genetics, University of Utah School of Medicine, Salt Lake City 84112, USA
    Circulation 115:3213-23. 2007
    ..This review discusses the latest findings in diabetic humans and in animal models and reviews emerging new mechanisms that may be involved in the development and progression of cardiac dysfunction in diabetes...
  2. ncbi Classification of the cardiomyopathies: a position statement from the European Society Of Cardiology Working Group on Myocardial and Pericardial Diseases
    Perry Elliott
    Hadassah University Hospital Ein Kerem, Kirjat Hadassah, Jerusalem 91120, Israel
    Eur Heart J 29:270-6. 2008
    ..three decades, heart muscle diseases have been classified into primary or idiopathic myocardial diseases (cardiomyopathies) and secondary disorders that have similar morphological appearances, but which are caused by an identifiable ..
  3. ncbi Diabetic cardiomyopathy: evidence, mechanisms, and therapeutic implications
    Zhi You Fang
    University of Queensland, Brisbane, 4012, Australia
    Endocr Rev 25:543-67. 2004
    ....
  4. ncbi Cardiovascular T2-star (T2*) magnetic resonance for the early diagnosis of myocardial iron overload
    L J Anderson
    Cardiovascular MR Unit, Royal Brompton Hospital, London, UK
    Eur Heart J 22:2171-9. 2001
    ..Early intensification of iron chelation therapy, guided by this technique, should reduce mortality from this reversible cardiomyopathy...
  5. pmc Desmin-related cardiomyopathy: an unfolding story
    Patrick M McLendon
    Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio 45229 3039, USA
    Am J Physiol Heart Circ Physiol 301:H1220-8. 2011
    ....
  6. ncbi Doxorubicin-induced cardiomyopathy from the cardiotoxic mechanisms to management
    Genzou Takemura
    Second Department of Internal Medicine, Gifu University School of Medicine, Gifu, Japan
    Prog Cardiovasc Dis 49:330-52. 2007
    ..We suggest this approach to preventing DOX-induced cardiomyopathy is worthy of serious consideration for clinical use...
  7. ncbi Myocardial steatosis is an independent predictor of diastolic dysfunction in type 2 diabetes mellitus
    Luuk J Rijzewijk
    Department of Endocrinology, VU University Medical Center, Amsterdam, The Netherlands
    J Am Coll Cardiol 52:1793-9. 2008
    ....
  8. ncbi Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functio
    Barry J Maron
    Circulation 113:1807-16. 2006
    ..A new contemporary and rigorous classification of cardiomyopathies (with definitions) is proposed here...
  9. ncbi Cardiogenetics, neurogenetics, and pathogenetics of left ventricular hypertrabeculation/noncompaction
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Postfach 20, 1180 Vienna, Austria, Europe
    Pediatr Cardiol 30:659-81. 2009
    ..This study aimed to review the current knowledge about the genetic or pathogenetic background of LVHT...
  10. pmc Non-invasive evaluation of the myocardial substrate of cardiac amyloidosis by gadolinium cardiac magnetic resonance
    E Perugini
    Institute of Cardiology, University of Bologna, S Orsola Malpighi Hospital, Bologna, Italy
    Heart 92:343-9. 2006
    ....
  11. ncbi Mouse model of desmin-related cardiomyopathy
    X Wang
    Divisions of Molecular Cardiovascular Biology, Children's Hospital Research Foundation, Cincinnati, Ohio, USA
    Circulation 103:2402-7. 2001
    ..However, the D7-des mutation is dominant negative, and expression of the mutant protein leads to the appearance of aggregates that are characteristic of and diagnostic for human desmin-related cardiomyopathy...
  12. ncbi T2-weighted cardiovascular magnetic resonance imaging
    Hassan Abdel-Aty
    Franz Volhard Klinik and Department of Radiology, Charite University Hospital, Berlin, Germany
    J Magn Reson Imaging 26:452-9. 2007
    ..Increased myocardial water content is an important feature of ischemic as well as nonischemic cardiomyopathies, which are often associated with acute myocardial inflammation...
  13. pmc Dysferlin-mediated membrane repair protects the heart from stress-induced left ventricular injury
    Renzhi Han
    Howard Hughes Medical Institute, The University of Iowa, Roy J and Lucille A Carver College of Medicine, Iowa City, IA 52242, USA
    J Clin Invest 117:1805-13. 2007
    ..Thus, our study establishes what we believe is a novel mechanism underlying the cardiomyopathy that results from a defective membrane repair in the absence of dysferlin...
  14. ncbi Diabetic cardiomyopathy--a distinct disease?
    Kaivan Khavandi
    Division of Cardiovascular and Endocrine Sciences, Core Technology Facility, University of Manchester, Manchester, UK
    Best Pract Res Clin Endocrinol Metab 23:347-60. 2009
    ....
  15. pmc Cardiomyopathy syndrome of atlantic salmon (Salmo salar L.) is caused by a double-stranded RNA virus of the Totiviridae family
    Oyvind Haugland
    Department of Basic Sciences and Aquatic Medicine, Norwegian School of Veterinary Science, Oslo, Norway
    J Virol 85:5275-86. 2011
    ..The identification of the causative agent for CMS is important for improved disease surveillance and disease control and will serve as a basis for vaccine development against the disease...
  16. ncbi Molecular basis of hereditary cardiomyopathy: abnormalities in calcium sensitivity, stretch response, stress response and beyond
    Akinori Kimura
    Department of Molecular Pathogenesis, Medical Research Institute, Tokyo Medical and Dental University, Japan
    J Hum Genet 55:81-90. 2010
    ....
  17. pmc Abnormal termination of Ca2+ release is a common defect of RyR2 mutations associated with cardiomyopathies
    Yijun Tang
    Department of Physiology and Pharmacology, The Libin Cardiovascular Institute of Alberta, University of Calgary, Calgary, AB, Canada
    Circ Res 110:968-77. 2012
    ..mutations in the cardiac ryanodine receptor (RyR2) have been associated with both cardiac arrhythmias and cardiomyopathies. It is clear that delayed afterdepolarization resulting from abnormal activation of sarcoplasmic reticulum ..
  18. ncbi Role of cardiac magnetic resonance imaging in the detection of cardiac amyloidosis
    Imran S Syed
    Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota 55905, USA
    JACC Cardiovasc Imaging 3:155-64. 2010
    ....
  19. pmc A novel totivirus and piscine reovirus (PRV) in Atlantic salmon (Salmo salar) with cardiomyopathy syndrome (CMS)
    Marie Løvoll
    Section for Virology and Serology, National Veterinary Institute, P, O, Box 750 Sentrum, 0106 Oslo, Norway
    Virol J 7:309. 2010
    ..In the present work we have studied whether PRV or other infectious agents may be involved in the etiology of CMS...
  20. ncbi Left ventricular non-compaction: insights from cardiovascular magnetic resonance imaging
    Steffen E Petersen
    University of Oxford Centre for Clinical Magnetic Resonance Research, Oxford, United Kingdom
    J Am Coll Cardiol 46:101-5. 2005
    ..LVNC) from lesser degrees of trabecular layering seen in healthy volunteers and, in those with cardiomyopathies and concentric left ventricular hypertrophy, potential differential diagnoses...
  21. ncbi Genetic counselling and testing in cardiomyopathies: a position statement of the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases
    Philippe Charron
    UPMC Univ Paris 6, AP HP, Hopital Pitie Salpetriere, Centre de Référence Maladies Cardiaques Héréditaires, Paris, France
    Eur Heart J 31:2715-26. 2010
    ..genetics present new opportunities and challenges for cardiologists who manage patients and families with cardiomyopathies. The aims of this position statement of the European Society of Cardiology Working Group on Myocardial and ..
  22. pmc Altered calcium homeostasis does not explain the contractile deficit of diabetic cardiomyopathy
    Lin Zhang
    Department of Physiology, Faculty of Medical and Health Sciences, University of Auckland, Auckland, New Zealand
    Diabetes 57:2158-66. 2008
    ..This study examines the extent to which the contractile deficit of diabetic cardiomyopathy is due to altered Ca(2+) homeostasis...
  23. ncbi Diabetes related cardiomyopathy time dependent echocardiographic evaluation in an experimental rat model
    M J Mihm
    Division of Pharmacology, College of Pharmacy, The Ohio State University, Columbus, USA
    Life Sci 69:527-42. 2001
    ..Furthermore, diastolic dysfunction precedes contractile abnormalities in the streptozotocin model, similar to events that occur in humans...
  24. ncbi Caveolin-3 knock-out mice develop a progressive cardiomyopathy and show hyperactivation of the p42/44 MAPK cascade
    Scott E Woodman
    Department of Molecular Pharmacology, Division of Hormone Dependent Tumor Biology, Albert Einstein College of Medicine, Bronx, New York 10461, USA
    J Biol Chem 277:38988-97. 2002
    ..Taken together, our data argue that loss of Cav-3 expression is sufficient to induce a molecular program leading to cardiac myocyte hypertrophy and cardiomyopathy...
  25. ncbi Senile systemic amyloidosis presenting with heart failure: a comparison with light chain-associated amyloidosis
    Belinda Ng
    Amyloidosis Treatment and Research Program, Sections of Cardiology, Boston University School of Medicine, Boston, MA, USA
    Arch Intern Med 165:1425-9. 2005
    ....
  26. ncbi Desmosomal dysfunction due to mutations in desmoplakin causes arrhythmogenic right ventricular dysplasia/cardiomyopathy
    Zhao Yang
    Department of Medicine, Baylor College of Medicine, Houston, TX 77030, USA
    Circ Res 99:646-55. 2006
    ..These data suggest DSP expression in cardiomyocytes is crucial for maintaining cardiac tissue integrity, and DSP abnormalities result in ARVD/C by cardiomyocyte death, changes in lipid metabolism, and defects in cardiac development...
  27. ncbi Regulation of hypertrophic and apoptotic signaling pathways by reactive oxygen species in cardiac myocytes
    Abdelkarim Sabri
    Department of Physiology and Biophysics, University of Alabama Birmingham, Birmingham, AL 35294, USA
    Antioxid Redox Signal 5:731-40. 2003
    ..enzymes that regulate their bioavailability are associated with cardiac hypertrophy and the pathogenesis of cardiomyopathies, myocardial infarction and heart failure...
  28. pmc Improved survival of thalassaemia major in the UK and relation to T2* cardiovascular magnetic resonance
    Bernadette Modell
    UCL Centre for Health Informatics and Multiprofessional Education, Holborn Union Building, Whittington Campus, Highgate Hill, London, N19 5LW, UK
    J Cardiovasc Magn Reson 10:42. 2008
    ..A study of survival and causes of death was undertaken which aimed to include the possible impact of T2* cardiovascular magnetic resonance (CMR)...
  29. pmc Autophagy and p62 in cardiac proteinopathy
    Qingwen Zheng
    Division of Basic Biomedical Sciences, Sanford School of Medicine of the University of South Dakota, 414 East Clark Street, Vermillion, SD 57069, USA
    Circ Res 109:296-308. 2011
    ..an important role of autophagy in protection against αB-crystallin-based (CryAB(R120G)) desmin-related cardiomyopathies (DRC), but this has not been demonstrated in a different model of cardiac proteinopathy...
  30. pmc Doxorubicin cardiomyopathy
    Kanu Chatterjee
    Department of Medicine, University of Iowa Carver College of Medicine, Iowa City, Iowa 52242 1081, USA kanu chatterjee uiowa edu
    Cardiology 115:155-62. 2010
    ..Extensive research has been done and is being done to discover preventive treatments. However an effective and clinically applicable preventive treatment is yet to be discovered...
  31. ncbi Cardiovascular magnetic resonance in cardiac amyloidosis
    Alicia Maria Maceira
    Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, UK
    Circulation 111:186-93. 2005
    ..Cardiac amyloidosis can be diagnostically challenging. Cardiovascular magnetic resonance (CMR) can assess abnormal myocardial interstitium...
  32. ncbi Desmin mutations as a cause of right ventricular heart failure affect the intercalated disks
    Ellen Otten
    Department of Genetics, University Medical Center Groningen, University of Groningen, The Netherlands
    Heart Rhythm 7:1058-64. 2010
    ..Right ventricular involvement including an arrhythmogenic right ventricular cardiomyopathy (ARVC)(-like) phenotype has occasionally been described in DES mutation-carrying patients...
  33. pmc Anthracycline cardiomyopathy is mediated by depletion of the cardiac stem cell pool and is rescued by restoration of progenitor cell function
    Antonella De Angelis
    Department of Experimental Medicine, Section of Pharmacology, Second Naples Medical School, Naples, Italy
    Circulation 121:276-92. 2010
    ....
  34. ncbi Myocardial fibrosis in isolated left ventricular non-compaction and its relation to disease severity
    Gaetano Nucifora
    Division of Cardiovascular Magnetic Resonance, Fondazione CNR Regione Toscana Gabriele Monasterio, Via Moruzzi 1, 56124 Pisa, Italy
    Eur J Heart Fail 13:170-6. 2011
    ..The aim of the present study was to evaluate the prevalence and extent of myocardial fibrosis in patients with isolated left ventricular non-compaction (LVNC) and to determine its relation to clinical status and LV systolic function...
  35. pmc MicroRNA-146a is a therapeutic target and biomarker for peripartum cardiomyopathy
    Julie Halkein
    Unit of Molecular Biology and Genetic Engineering, GIGA, University of Liege, Liege, Belgium
    J Clin Invest 123:2143-54. 2013
    ..These results demonstrate that miR-146a is a downstream-mediator of 16K PRL that could potentially serve as a biomarker and therapeutic target for PPCM...
  36. ncbi Diabetic cardiomyopathy: the search for a unifying hypothesis
    Indu G Poornima
    Department of Medicine, Allegheny General Hospital, Pittsburgh, PA 15212, USA
    Circ Res 98:596-605. 2006
    ..The recent recognition that diabetes involves more than abnormal glucose homeostasis provides important new opportunities to examine and understand the impact of complex metabolic disturbances on cardiac structure and function...
  37. ncbi Extent of left ventricular scar predicts outcomes in ischemic cardiomyopathy patients with significantly reduced systolic function: a delayed hyperenhancement cardiac magnetic resonance study
    Deborah H Kwon
    Department of Cardiovascular Medicine, Cleveland Clinic, Cleveland, Ohio 44195, USA
    JACC Cardiovasc Imaging 2:34-44. 2009
    ....
  38. ncbi Molecular genetics of arrhythmogenic right ventricular cardiomyopathy: emerging horizon?
    J Peter van Tintelen
    Departments of Genetics, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands
    Curr Opin Cardiol 22:185-92. 2007
    ..Recent developments in the elucidation of genes underlying arrhythmogenic right ventricular cardiomyopathy and possible pathogenic mechanisms will be highlighted...
  39. ncbi Type 1 diabetic cardiomyopathy in the Akita (Ins2WT/C96Y) mouse model is characterized by lipotoxicity and diastolic dysfunction with preserved systolic function
    Ratnadeep Basu
    Department of Physiology, Rm 474, Heritage Medical Research Centre, University of Alberta, Edmonton, Alberta T6G 2S2, Canada
    Am J Physiol Heart Circ Physiol 297:H2096-108. 2009
    ..We conclude that early type 1 diabetic cardiomyopathy is characterized by diastolic dysfunction associated with lipotoxic cardiomyopathy with preserved systolic function in the absence of interstitial fibrosis and hypertrophy...
  40. ncbi Thromboxane A2 mediates iron-overload cardiomyopathy in mice through calcineurin-nuclear factor of activated T cells signaling pathway
    Heng Lin
    Institute of Physiology, College of Medicine, Taipei Medical University
    Circ J 77:2586-95. 2013
    ....
  41. ncbi Epidemiology and clinical management of cardiomyopathies and heart failure in China
    H Jiang
    Department of Cardiology, Zhongshan Hospital, Shanghai Medical College, Fudan University, Shanghai 200032, China
    Heart 95:1727-31. 2009
    ..In this review the epidemiological data on cardiomyopathies, HF and current treatment for chronic HF among people living in China are summarised.
  42. ncbi Systemic cardiac amyloidoses: disease profiles and clinical courses of the 3 main types
    Claudio Rapezzi
    Istituto di Cardiologia, Policlinico S Orsola Malpighi, Via Massarenti n 9, 40125 Bologna, Italy
    Circulation 120:1203-12. 2009
    ..In this study, we compared the diagnostic/clinical profiles of these 3 types of systemic cardiac amyloidosis...
  43. pmc SGLT1, a novel cardiac glucose transporter, mediates increased glucose uptake in PRKAG2 cardiomyopathy
    Sanjay K Banerjee
    Cardiovascular Institute, University of Pittsburgh, Pittsburgh, PA 15213 2582, USA
    J Mol Cell Cardiol 49:683-92. 2010
    ..Increased AMPK activity leads to upregulation of SGLT1, which in turn mediates increased cardiac glucose uptake...
  44. ncbi Chemical cardiomyopathies: the negative effects of medications and nonprescribed drugs on the heart
    Vincent M Figueredo
    Einstein Institute for Heart and Vascular Health, Albert Einstein Medical Center, Jefferson Medical College, Philadelphia, PA, USA
    Am J Med 124:480-8. 2011
    ..Pathophysiologic mechanisms underlying the development of chemical-induced cardiomyopathies vary depending on the inciting agent, including direct toxic effects, neurohormonal activation, altered ..
  45. ncbi Myocardial steatosis, cardiac remodelling and fitness in insulin-sensitive and insulin-resistant obese women
    Wolfgang Utz
    Working Group Cardiac MR Medical Faculty of the Charité Campus Buch and HELIOS Klinikum Berlin Buch, Berlin, Germany
    Heart 97:1585-9. 2011
    ..Lipotoxic injury from altered myocardial metabolism may be causative. Whether such association also exists in obese, non-diabetic women is unknown...
  46. pmc The sarcomeric Z-disc and Z-discopathies
    Ralph Knoll
    Centre for Research Excellence, British Heart Foundation, National Heart and Lung Institute, Imperial College, South Kensington Campus, Flowers Building, 4th Floor, London SW72AZ, UK
    J Biomed Biotechnol 2011:569628. 2011
    ..This paper provides a brief overview of these novel aspects as well as points to future research directions...
  47. ncbi Desmin myopathy, a skeletal myopathy with cardiomyopathy caused by mutations in the desmin gene
    M C Dalakas
    Neuromuscular Diseases Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892 1382, USA
    N Engl J Med 342:770-80. 2000
    ..Skeletal and cardiac myopathy develops in mice that lack desmin, suggesting that mutations in the desmin gene may be pathogenic...
  48. ncbi Cardiac connexins, mutations and arrhythmias
    Mario Delmar
    The Leon H Charney Division of Cardiology, New York University School of Medicine, New York, New York 10016, USA
    Curr Opin Cardiol 27:236-41. 2012
    ..However, the relation between nucleotide substitutions in the connexin gene and the occurrence of cardiac arrhythmias remains largely unexplored...
  49. ncbi The predictive values of beta1-adrenergic and M2 muscarinic receptor autoantibodies for sudden cardiac death in patients with chronic heart failure
    Juanhui Pei
    State Key Laboratory of Cardiovascular Disease, Fuwai Hospital and Cardiovascular Institute, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
    Eur J Heart Fail 14:887-94. 2012
    ..The goal of this project is to evaluate whether beta1-AAbs and M2-AAbs predict prognosis and sudden cardiac death (SCD) in CHF...
  50. pmc Diagnostic and prognostic value of cardiovascular magnetic resonance in non-ischaemic cardiomyopathies
    Chirine Parsai
    Cardiovascular Magnetic Resonance Unit, Royal Brompton and Harefield NHS Trust, London, UK
    J Cardiovasc Magn Reson 14:54. 2012
    ..The potential application of CMR in the clinical approach of a patient with suspected non-ischaemic cardiomyopathy is discussed in this review...
  51. pmc Haploinsufficiency of target of rapamycin attenuates cardiomyopathies in adult zebrafish
    Yonghe Ding
    Department of Biochemistry and Molecular Biology, Mayo Clinic College of Medicine, Rochester, MN 55905, USA
    Circ Res 109:658-69. 2011
    ..We explored adult zebrafish as a novel vertebrate model for dissecting signaling pathways in cardiomyopathy...
  52. pmc Desminopathies: pathology and mechanisms
    Christoph S Clemen
    Institute of Biochemistry I, Medical Faculty, University of Cologne, Joseph Stelzmann Street 52, 50931 Cologne, Germany
    Acta Neuropathol 125:47-75. 2013
    ..desmin gene on chromosome 2q35 cause autosomal dominant, autosomal recessive, and sporadic myopathies and/or cardiomyopathies with marked phenotypic variability. The disease onset ranges from childhood to late adulthood...
  53. ncbi Comparison of magnetic resonance feature tracking for strain calculation with harmonic phase imaging analysis
    Kan N Hor
    Department of Cardiology, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio, USA
    JACC Cardiovasc Imaging 3:144-51. 2010
    ....
  54. ncbi Adipophilin/perilipin-2 as a lipid droplet-specific marker for metabolically active cells and diseases associated with metabolic dysregulation
    Beate Katharina Straub
    Department of General Pathology, Institute of Pathology, Heidelberg, Germany
    Histopathology 62:617-31. 2013
    ..We evaluated the value of adipophilin immunohistochemistry for the diagnosis of diseases associated with LD accumulation...
  55. pmc Iron overload cardiomyopathy: better understanding of an increasing disorder
    Pradeep Gujja
    Division of Cardiovascular Diseases, Department of Internal Medicine, University of Cincinnati, Cincinnati, Ohio 45267 0542, USA
    J Am Coll Cardiol 56:1001-12. 2010
    ..There is a need for the development of clinical guidelines in order to improve the management of this emerging complex disease...
  56. ncbi Quantification of left ventricular noncompaction and trabecular delayed hyperenhancement with cardiac MRI: correlation with clinical severity
    Jonathan D Dodd
    Department of Radiology, Massachusetts General Hospital and Harvard Medical School, Boston, MA 02114, USA
    AJR Am J Roentgenol 189:974-80. 2007
    ....
  57. ncbi Causes of death in pedigrees with the 3243A>G mutation in mitochondrial DNA
    K Majamaa-Voltti
    Department of Internal Medicine, University of Oulu, P O Box 5000, FIN 90014 Oulu, Finland
    J Neurol Neurosurg Psychiatry 79:209-11. 2008
    ..Causes of death of patients with the 3243A>G mutation have been described in case reports or case series with a limited number of subjects...
  58. pmc Transcriptome profiling of immune responses to cardiomyopathy syndrome (CMS) in Atlantic salmon
    Gerrit Timmerhaus
    Nofima AS, As, Norway
    BMC Genomics 12:459. 2011
    ..Here we present the first characterisation of host immune responses to CMS assessed by microarray transcriptome profiling...
  59. pmc A murine model of isolated cardiac steatosis leads to cardiomyopathy
    DENIS J GLENN
    Department of Medicine and Diabetes Center, University of California at San Francisco, San Francisco, CA 94143 0540, USA
    Hypertension 57:216-22. 2011
    ..These findings suggest that triglyceride accumulation, in the absence of systemic metabolic derangement, results in cardiac dysfunction and decreased mitochondrial biogenesis...
  60. ncbi Clinical characteristics of peripartum cardiomyopathy in the United States: diagnosis, prognosis, and management
    Uri Elkayam
    Division of Cardiology, Department of Medicine, University of Southern California Keck School of Medicine, Los Angeles, California, USA
    J Am Coll Cardiol 58:659-70. 2011
    ..The purposes of this review are to describe the clinical profile of peripartum cardiomyopathy in the United States and to provide recommendations for the diagnosis and the management of this disease...
  61. ncbi Inhibition of gene expression of heart fatty acid binding protein and organic cation/carnitine transporter in doxorubicin cardiomyopathic rat model
    Mohamed M Sayed-Ahmed
    Department of Pharmacology, College of Pharmacy, King Saud University, P O Box 2457, Riyadh 11451, Saudi Arabia
    Eur J Pharmacol 640:143-9. 2010
    ..The progressive increase in cardiotoxicity enzymatic indices and the decrease in H-FABP and OCTN2 expression may point to the possible contribution of H-FABP and OCTN2 as a mechanism during development of doxorubicin cardiotoxicity...
  62. ncbi Thrombopoietin protects against in vitro and in vivo cardiotoxicity induced by doxorubicin
    Karen Li
    Department of Pediatrics, The Chinese University of Hong Kong, Shatin, NT, Hong Kong
    Circulation 113:2211-20. 2006
    ..Our hypothesis is that the hematopoietic/megakaryocytopoietic growth factor thrombopoietin (TPO) protects against DOX-induced cardiotoxicity and might involve antiapoptotic mechanism exerted on cardiomyocytes...
  63. ncbi Evaluation of the clinical relevance of baseline left ventricular ejection fraction as a predictor of recovery or persistence of severe dysfunction in women in the United States with peripartum cardiomyopathy
    Sorel Goland
    Division of Cardiovascular Medicine, Department of Medicine, Keck School of Medicine, University of Southern California, Los Angeles, California, USA
    J Card Fail 17:426-30. 2011
    ..Baseline left ventricular ejection fraction (LVEF) has been shown to be associated with likelihood of recovery in patients with peripartum cardiomyopathy (PPCM). The clinical relevance of this association for individual patients is unclear...
  64. ncbi Magnetic resonance imaging of myocardial injury and ventricular torsion after marathon running
    Henner Hanssen
    Department of Prevention and Sports Medicine, Klinikum rechts der Isar, Technische Universitat Munchen, Munich, Germany
    Clin Sci (Lond) 120:143-52. 2011
    ..The stress of marathon running seems to be better described as a burden of myocardial overstimulation rather than cardiac injury...
  65. ncbi Myocardial delayed enhancement by magnetic resonance imaging in patients with muscular dystrophy
    Marly Conceição Silva
    Axial Centro de Imagem, Belo Horizonte, Minas Gerais, Brazil
    J Am Coll Cardiol 49:1874-9. 2007
    ..This study sought to analyze whether cardiovascular magnetic resonance (CMR) can detect and quantify myocardial damage in the early stages of cardiomyopathy in muscular dystrophies (MD)...
  66. pmc Losartan decreases cardiac muscle fibrosis and improves cardiac function in dystrophin-deficient mdx mice
    Christopher F Spurney
    Division of Cardiology, Children s National Medical Center, Washington, DC, USA
    J Cardiovasc Pharmacol Ther 16:87-95. 2011
    ..Chronic treatment with losartan decreases cardiac and skeletal muscle fibrosis and improves cardiac systolic function in dystrophin-deficient mdx mice...
  67. ncbi Mechanisms and management of doxorubicin cardiotoxicity
    Y Shi
    Division of Cardiology, Heart and Stroke Richard Lewar Centre of Excellence, University Health Network, University of Toronto, Toronto General Hospital, Ontario, Canada
    Herz 36:296-305. 2011
    ..Novel approaches such as anti-miR-146 or recombinant NRG1 to increase cardiomyocyte resistance to toxicity may be of interest in the future...
  68. ncbi Clinical spectrum, morbidity, and mortality in 113 pediatric patients with mitochondrial disease
    Fernando Scaglia
    Department of Molecular and Human Genetics, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA
    Pediatrics 114:925-31. 2004
    ..The aim of this study was to elucidate the frequency of major clinical manifestations in children with mitochondrial disease and establish their clinical course, prognosis, and rates of survival depending on their clinical features...
  69. ncbi Mice lacking calsarcin-1 are sensitized to calcineurin signaling and show accelerated cardiomyopathy in response to pathological biomechanical stress
    Norbert Frey
    Department of Molecular Biology, University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, Texas 75390 9148, USA
    Nat Med 10:1336-43. 2004
    ..a family of muscle-specific proteins of the sarcomeric Z-disc, a focal point in the pathogenesis of human cardiomyopathies. We show that calsarcin-1 negatively modulates the functions of calcineurin, such that calcineurin signaling ..
  70. ncbi Monolysocardiolipins accumulate in Barth syndrome but do not lead to enhanced apoptosis
    Fredoen Valianpour
    Laboratory of Genetic Metabolic Diseases, Department of Clinical Chemistry, Emma Children s Hospital, Academic Medical Center, University of Amsterdam, 1100 DE Amsterdam, The Netherlands
    J Lipid Res 46:1182-95. 2005
    ....
  71. pmc Laminopathies and the long strange trip from basic cell biology to therapy
    Howard J Worman
    Department of Medicine, Columbia University College of Physicians and Surgeons, 630 West 168th Street, New York, NY 10032, USA
    J Clin Invest 119:1825-36. 2009
    ..Here, we review the laminopathies and the long strange trip from basic cell biology to therapeutic approaches for these diseases...
  72. ncbi Remodeling of gap junctions and slow conduction in a mouse model of desmin-related cardiomyopathy
    Joseph J Gard
    Department of Pathology, Box 8118, Washington University School of Medicine, 660 South Euclid Avenue, St Louis, MO 63110, USA
    Cardiovasc Res 67:539-47. 2005
    ....
  73. ncbi Role of L-type Ca2+ channels in iron transport and iron-overload cardiomyopathy
    Gavin Y Oudit
    Heart and Stroke Richard Lewar Centre of Excellence, University Health Network, University of Toronto, Ontario, M5S 3E2, Canada
    J Mol Med (Berl) 84:349-64. 2006
    ..Consequently, LTCC blockers represent a potential new therapy to reduce the toxic effects of excess iron...
  74. ncbi Apical ballooning syndrome or takotsubo cardiomyopathy: a systematic review
    Monica Gianni
    Department of Clinical Medicine, University of Insubria, Varese, Italy
    Eur Heart J 27:1523-9. 2006
    ..To clarify the major features of the apical ballooning syndrome, we performed a systematic review of the existing literature...
  75. ncbi Null mutation in the desmin gene gives rise to a cardiomyopathy
    L Thornell
    Department of Anatomy, Umea University, Umea, Sweden
    J Mol Cell Cardiol 29:2107-24. 1997
    ..Thus, the intermediate filaments are required to maintain the basic integrity of cardiomyocytes and especially the link between the intermediate filaments and the sarcolemma appear more important than previously realized...
  76. ncbi Novel imaging techniques for diffuse myocardial fibrosis
    Daniel M Sado
    Department of Inherited Cardiac Disease, The Heart Hospital, 16 18 Westmoreland Street, London, W1G 8PH, UK
    Future Cardiol 7:643-50. 2011
    ..This review explores the basic biology of DMF and then discusses the ability of various cardiac imaging modalities to evaluate this variable, speculating on how this area of research may develop over the next few years...
  77. ncbi [An adult case of congenital fiber type disproportion (CFTD) with cardiomyopathy]
    Kengo Fujita
    Department of Neurology, Kansai Medical University
    Rinsho Shinkeigaku 45:380-2. 2005
    ..He was diagnosed as having CFTD because there were no histochemical abnormalities which characterize other congenital myopathies except for the type 1 predominancy and atrophy...
  78. pmc Increased connective tissue growth factor associated with cardiac fibrosis in the mdx mouse model of dystrophic cardiomyopathy
    Carol G Au
    Kids Heart Research and Institute for Neuroscience and Muscle Research, The Children s Hospital at Westmead, Sydney, Australia
    Int J Exp Pathol 92:57-65. 2011
    ..In summary, an association was observed between cardiac fibrosis and increased CTGF expression in the mdx mouse heart. CTGF may be a key mediator of early and persistent fibrosis in dystrophic cardiomyopathy...
  79. ncbi Current state of knowledge on aetiology, diagnosis, management, and therapy of peripartum cardiomyopathy: a position statement from the Heart Failure Association of the European Society of Cardiology Working Group on peripartum cardiomyopathy
    Karen Sliwa
    Hatter Cardiovascular Research Institute, University of Cape Town, Cape Town, South Africa
    Eur J Heart Fail 12:767-78. 2010
    ..Oxidative stress and the generation of a cardiotoxic subfragment of prolactin may play key roles in the pathophysiology of PPCM. In this regard, pharmacological blockade of prolactin offers the possibility of a disease-specific therapy...
  80. ncbi Reperfusion haemorrhage as determined by cardiovascular MRI is a predictor of adverse left ventricular remodelling and markers of late arrhythmic risk
    Adam N Mather
    Division of Cardiovascular and Neuronal Remodelling, University of Leeds, G Floor, Jubilee Wing, Leeds General Infirmary, Great George Street, Leeds, UK
    Heart 97:453-9. 2011
    ..The clinical implications of myocardial haemorrhage following primary percutaneous coronary intervention (PPCI) remain undetermined...
  81. ncbi Infarct tissue heterogeneity assessed with contrast-enhanced MRI predicts spontaneous ventricular arrhythmia in patients with ischemic cardiomyopathy and implantable cardioverter-defibrillator
    Stijntje D Roes
    Department of Radiology, Leiden University Medical Center, Leiden, The Netherlands
    Circ Cardiovasc Imaging 2:183-90. 2009
    ....
  82. ncbi Coronary microvascular dysfunction
    Paolo G Camici
    Medical Research Council Clinical Sciences Centre Hammersmith Hospital, and National Heart and Lung Institute, Imperial College, London, United Kingdom
    N Engl J Med 356:830-40. 2007
  83. pmc Circumferential strain analysis identifies strata of cardiomyopathy in Duchenne muscular dystrophy: a cardiac magnetic resonance tagging study
    Kan N Hor
    Department of Cardiology, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio 45229 3039, USA
    J Am Coll Cardiol 53:1204-10. 2009
    ..This study sought to evaluate the natural history of occult cardiac dysfunction in Duchenne muscular dystrophy (DMD)...
  84. pmc Diabetic cardiomyopathy in Zucker diabetic fatty rats: the forgotten right ventricle
    Charissa E van den Brom
    Department of Internal Medicine Diabetes Centre, VU University Medical Centre, Amsterdam, The Netherlands
    Cardiovasc Diabetol 9:25. 2010
    ..The purpose of the present study was to investigate whether LV changes are paralleled by RV alterations in a rat model of diabetes...
  85. pmc ALP/Enigma PDZ-LIM domain proteins in the heart
    Ming Zheng
    Institute of Molecular Medicine, Peking University, Beijing 100871, China
    J Mol Cell Biol 2:96-102. 2010
    ..This review will focus on recent advances in the biological roles of ALP/Enigma PDZ-LIM domain proteins in cardiac muscle and provide insights into mechanisms by which mutations in these proteins are related to human cardiac disease...
  86. pmc A common MYBPC3 (cardiac myosin binding protein C) variant associated with cardiomyopathies in South Asia
    Perundurai S Dhandapany
    Department of Biochemistry, Madurai Kamaraj University, Madurai 625 021, India
    Nat Genet 41:187-91. 2009
    ..However, its genetic etiology remains largely unknown. Cardiomyopathies due to sarcomeric mutations are a major monogenic cause for heart failure (MIM600958)...
  87. ncbi Mutations in sarcomere protein genes in left ventricular noncompaction
    Sabine Klaassen
    Max Delbruck Center for Molecular Medicine, Robert Roessle Strasse 10, 13092 Berlin, Germany
    Circulation 117:2893-901. 2008
    ..We speculated that mutations in sarcomere protein genes known to cause hypertrophic cardiomyopathy and dilated cardiomyopathy may be associated with left ventricular noncompaction...
  88. ncbi Desmoglein-2 mutations in arrhythmogenic right ventricular cardiomyopathy: a genotype-phenotype characterization of familial disease
    Petros Syrris
    Department of Medicine, The Heart Hospital, University College London and University College London Hospitals Trust, 16 18 Westmoreland Street, London W1G 8PH, UK
    Eur Heart J 28:581-8. 2007
    ..In this study, we aimed to clinically characterize probands and family members carrying a DSG2 mutation...
  89. ncbi Phospholipid abnormalities in children with Barth syndrome
    Michael Schlame
    Department of Anesthesiology, New York University School of Medicine, New York, New York 10016, USA
    J Am Coll Cardiol 42:1994-9. 2003
    ..We sought to identify characteristic lipid abnormalities in patients with Barth syndrome (BTHS) and to correlate the lipid profile to phenotype and genotype...
  90. ncbi Clinical characterization of left ventricular noncompaction in children: a relatively common form of cardiomyopathy
    Ricardo H Pignatelli
    Lillie Frank Abercrombie Division of Pediatric Cardiology, Texas Children s Hospital, 6621 Fannin, Houston 77030, Tex, USA
    Circulation 108:2672-8. 2003
    ..The purpose of this study was to identify the clinical characteristics of children with LVNC...
  91. ncbi Diabetes and the accompanying hyperglycemia impairs cardiomyocyte calcium cycling through increased nuclear O-GlcNAcylation
    Raymond J Clark
    Department of Medicine, University of California San Diego, La Jolla, California 92093, USA
    J Biol Chem 278:44230-7. 2003
    ..Our results suggest that the hexosamine pathway, and O-GlcNAcylation in particular, is important in impaired cardiac myocyte function and the development of diabetic cardiomyopathy...
  92. ncbi Evaluation of the accuracy of gadolinium-enhanced cardiovascular magnetic resonance in the diagnosis of cardiac sarcoidosis
    Jan Peter Smedema
    Department of Cardiology, University Hospital Maastricht, Maastricht, The Netherlands
    J Am Coll Cardiol 45:1683-90. 2005
    ..This study analyzed the accuracy of gadolinium-enhanced cardiovascular magnetic resonance (CMR) for the diagnosis of cardiac sarcoidosis (CS)...
  93. pmc Resveratrol, an activator of SIRT1, upregulates sarcoplasmic calcium ATPase and improves cardiac function in diabetic cardiomyopathy
    M Sulaiman
    Dept of Physiology and Biophysics, Univ of Illinois, Chicago, USA
    Am J Physiol Heart Circ Physiol 298:H833-43. 2010
    ....
  94. ncbi Role of interleukin 6 in myocardial dysfunction of meningococcal septic shock
    Nazima Pathan
    Department of Paediatrics, Imperial College of Science, Technology and Medicine, W2 1PG, London, UK
    Lancet 363:203-9. 2004
    ..During the sepsis-induced inflammatory process, specific factors are released that depress myocardial contractile function. We aimed to identify these mediators of myocardial depression in meningococcal septic shock...
  95. ncbi Correlation between serum levels of cardiac troponin-T and the severity of the chronic cardiomyopathy induced by doxorubicin
    E H Herman
    Division of Applied Pharmacology Research HFD 910, Center for Drug Evaluation and Research, Food and Drug Administration, Laurel, MD 20708, USA
    J Clin Oncol 17:2237-43. 1999
    ..To investigate, over a wide range of cumulative doxorubicin doses, the feasibility of using serum concentrations of cardiac troponin-T (cTnT) as a biomarker for doxorubicin-induced myocardial damage...
  96. ncbi Transgenic mice overexpressing mutant PRKAG2 define the cause of Wolff-Parkinson-White syndrome in glycogen storage cardiomyopathy
    Michael Arad
    Department of Genetics, Harvard Medical School and Howard Hughes Medical Institute, 200 Longwood Ave, Boston, Mass 02115, USA
    Circulation 107:2850-6. 2003
    ..Pathological examinations of affected human hearts reveal vacuoles containing amylopectin, a glycogen-related substance...
  97. ncbi Clinical and molecular findings in children with complex I deficiency
    M Bugiani
    Department of Child Neurology, National Institute of Neurology C Besta, Milano, Italy
    Biochim Biophys Acta 1659:136-47. 2004
    ..The latter cases suggest the possibility of unconventional patterns of inheritance in complex I defects...
  98. ncbi Missense mutations in desmin associated with familial cardiac and skeletal myopathy
    L G Goldfarb
    Medical Neurology Branch, National Institute of Neurological Disorders and Stroke, NIH, Bethesda, Maryland 20892, USA
    Nat Genet 19:402-3. 1998
    ..Compound heterozygosity for two other mutations, A360P and N393I, was detected in a second family characterized by childhood-onset aggressive course of cardiac and skeletal myopathy...
  99. ncbi Human amyloidogenic light chains directly impair cardiomyocyte function through an increase in cellular oxidant stress
    Daniel A Brenner
    Whitaker Cardiovascular Institute and Amyloid Treatment and Research Program, Evans Department of Medicine, Boston University School of Medicine, Boston, Mass 02118, USA
    Circ Res 94:1008-10. 2004
    ..Antioxidant therapies or treatment strategies aimed at eliminating circulating LC proteins may therefore be beneficial in the treatment of this fatal disease...
  100. ncbi A series of Chinese patients with desminopathy associated with six novel and one reported mutations in the desmin gene
    D Hong
    Department of Neurology, Peking University First Hospital, Beijing Department of Neurology, Huashan Hospital, Fudan University, Shanghai, China
    Neuropathol Appl Neurobiol 37:257-70. 2011
    ..Desminopathy is a hereditary cardiac and skeletal myopathy caused by mutations in the desmin gene. This study summarizes the clinical, myopathological and genetic features of a series of Chinese patients with desminopathy...
  101. ncbi Malignant and benign mutations in familial cardiomyopathies: insights into mutations linked to complex cardiovascular phenotypes
    Qian Xu
    Department of Neurobiology, Physiology and Behavior, University of California, Davis, One Shields Avenue, CA 95616, USA
    J Mol Cell Cardiol 48:899-909. 2010
    b>Cardiomyopathies, familial or sporadic, have become recognized as one of the leading cardiac threats. Hypertrophic cardiomyopathy (HCM) affects 0.2% of the population and is the leading cause of sudden death in young adults...

Research Grants73

  1. Sphingolipid Metabolism in Drosophila Development
    Julie D Saba; Fiscal Year: 2013
    ..Excessive autophagy underlies many human disease states including neurodegenerative diseases, cardiomyopathies, cancer, programmed cell death, and bacterial and viral infections...
  2. Targeting Cell Death to Prevent Ventricular Remodeling and Heart Failure
    Abhinav Diwan; Fiscal Year: 2011
    ..If the stated hypothesis is proved to be true, it will engender a paradigm shift change our approach to cardiomyopathies from stress-centricism, to one based on a targeting common pathophysiological events encompassing both ..
  3. Novel Regenerative Therapy for Myocardial Infarction
    Andrew R Mendelsohn; Fiscal Year: 2011
    ..Prior work has shown that NRG1b1 effectively treated ischemic, dilated, and viral cardiomyopathies in mouse, rat and dog model systems...
  4. Mechanisms of coronary microvascular dysfunction in metabolic syndrome
    Johnathan D Tune; Fiscal Year: 2012
    ..and mortality to many cardiovascular diseases including: hypertension, stroke, coronary artery disease, cardiomyopathies, myocardial infarction, congestive heart failure, and sudden cardiac death...
  5. Arrhythmia Mechanisms in Sarcomeric Cardiomyopathies
    Bjorn C Knollmann; Fiscal Year: 2012
    ..in the intact heart The results of the proposed experiments will significantly advance our understanding of arrhythmia mechanisms responsible for the high rate of sudden cardiac death in sarcomeric cardiomyopathies.
  6. Innate Immunity and Cardiovascular Function
    Gregory L Stahl; Fiscal Year: 2010
    ..Mice deficient in the innate immune molecule mannose binding lectin are protected from hyperglycemia-induced cardiac fibrogenesis, vascular dysfunction and associated cardiomyopathies.
  7. Determinants of Disease Expression in Arrhythmogenic Cardiomyopathy
    Jeffrey E Saffitz; Fiscal Year: 2010
    ..The rate of appropriate ICD shocks in patients with ARVC greatly exceeds that for other cardiomyopathies and the ion channelopathies...
  8. Identification and Validation of Alcohol Biomarker Signatures by Proteomics
    Emanuel Rubin; Fiscal Year: 2010
    ..patterns for validation, integrate data in a relational database for future comparative studies with other cardiomyopathies, and map clusters of protein changes in affected pathways of the protein interactome in order to develop a ..
  9. Cardiomyocyte Zinc and its Regulation of Contraction-Relaxation Function
    Bradley M Palmer; Fiscal Year: 2012
    ..such as diabetes, hypertension coronary artery disease or myocardial infarction) that lead to related cardiomyopathies or heart failure...
  10. Cullin mediated protein turnover in skeletal and cardiac muscles
    Stephan Lange; Fiscal Year: 2012
    ..like the limb-girdle muscular dystrophy (type 2J) through links with obscurin proteins, but also for cardiomyopathies, since cullin proteins, and some of their binding partners are significantly altered in animal models for ..
  11. INTERACTIONS BETWEEN INTERMEDIATE FILAMENTS AND NUCLEUS
    Robert D Goldman; Fiscal Year: 2013
    ..Mutations in the nuclear lamins cause a variety of diseases including Emery-Dreifuss Muscular Dystrophy, cardiomyopathies, lipodystrophies and Hutchinson-Gilford Progeria Syndrome...
  12. The S1P1 receptor regulates sarcomere assembly and cardiac development
    Roland S Wu; Fiscal Year: 2013
    ..Defects in cardiac development lead to a wide variety of pathology, including congenital heart defects, cardiomyopathies, and valvular diseases...
  13. The Role of the Proteasome in Troponin related Cardiomyopathies
    ALDRIN VERNO GOMES; Fiscal Year: 2013
    ..provided by applicant): Despite significant advances in diagnosis and treatment of heart diseases, cardiomyopathies remain the most prevalent cause of death in developed countries...
  14. The Regulation of Sarcomeric Function by Tropomyosin
    David F Wieczorek; Fiscal Year: 2010
    ..This is important for biomedical research since mutations in TM cause hypertrophic and dilated cardiomyopathies that can lead to heart failure...
  15. Human serum carbonyl proteome in cardiovascular diseases
    Nisha Jain Garg; Fiscal Year: 2010
    ..for engineering the combination therapies for the prevention and treatment of chagasic, and possibly other cardiomyopathies. PUBLIC RELEVANCE: The studies in this project are aimed at developing the Chagas disease-associated plasma ..
  16. Physical and Functional Systems Analysis of Mammalian Mitochondria
    JODI M NUNNARI; Fiscal Year: 2013
    ..emerged as causative or contributing factors in a growing number of diverse human diseases such as cancer, cardiomyopathies, metabolic syndrome, and various neurodegenerative disorders...
  17. Cullin mediated protein turnover in skeletal and cardiac muscles
    Stephan Lange; Fiscal Year: 2013
    ..like the limb-girdle muscular dystrophy (type 2J) through links with obscurin proteins, but also for cardiomyopathies, since cullin proteins, and some of their binding partners are significantly altered in animal models for ..
  18. High throughput screening assays to identify small molecules that target the ClpX
    Carolyn K Suzuki; Fiscal Year: 2010
    ..clinical targets in mitochondrial dysfunction, which is associated with numerous neuromuscular disorders, cardiomyopathies, cancers and aging...
  19. HTS for Regulated Muscle Thin Filament Function.
    JAMES DOUGLAS POTTER; Fiscal Year: 2010
    A hallmark of sarcomeric gene mutations in cardiomyopathies is their ability to alter the calcium regulation of cardiac muscle contraction...
  20. The Role of Cardiomyopathic Troponin C Mutations in Skeletal and Cardiac Muscle C
    Jose Renato Pinto; Fiscal Year: 2011
    ..the primary Ca2+ sensor of striated muscle and has been a target of Hypertrophic (HCM) and Dilated (DCM) Cardiomyopathies. HCM or DCM are genetic disorders caused by the mutations in the TnC gene that are characterized by ..
  21. Muscle-Resident Stem Cells for Angiogenesis and Vascular Maturation in PAD
    Christopher D Kontos; Fiscal Year: 2013
    ..loss of Bag3 in mice results in a fulminant myopathy, and mutation in human BAG3 has been linked to inherited cardiomyopathies. We investigated Bag3's effects in limb ischemia after it was identified as a component of the limb ..
  22. Reductive Stress Induces Proteotoxic Cardiac Disease
    RAJASEKARAN NAMAKKAL SOORAPPAN; Fiscal Year: 2013
    ..Mounting evidence indicates that a majority of protein aggregation cardiomyopathies (PAC) caused by mutations in cytoskeletal proteins or chaperones involve pre-amyloid aggregates, oxidative ..
  23. Mechanisms of Regulation Of NHE-1
    John R Raymond; Fiscal Year: 2012
    ..The work could lead to new therapies for hypertension, congestive heart failure, cardiomyopathies and kidney failure.
  24. Integrative Approach to Divergent Remodeling in Thin Filament Cardiomyopathies
    Jil C Tardiff; Fiscal Year: 2013
    ..C- terminal "unstructured" domain and determine the pathogenic mechanism underlying the severe, progressive cardiomyopathies caused by mutations in this region...
  25. LAMIN A PROGERIA MUTATIONS AND NUCLEAR FUNCTION
    Robert D Goldman; Fiscal Year: 2010
    ..the mechanisms responsible for the multiple age-related disorders seen in patients with progeria, including cardiomyopathies and strokes...
  26. CARDIAC CONTRACTILE KINETICS
    Michael Regnier; Fiscal Year: 2009
    ..novel information regarding activation processes in cardiac muscle that allow fine-tuning of contractility at the cellular level in response to beat-to-beat alterations in venous return that can fail in cardiomyopathies.
  27. Cardiac Lipotoxicity and Ceramide Metabolism in Heart Failure
    P Christian Schulze; Fiscal Year: 2013
    ..In myocardium from patients with ischemic and non-ischemic cardiomyopathies and non- diseased controls, we will analyze the myocardial lipid composition of ceramides, diacylglycerides, ..
  28. Vevo 2100 Imaging System - High Resolution Ultrasound for Biomicroscopy
    R John Solaro; Fiscal Year: 2013
    ..as: (1) maladaptive post-translational modifications and mutations linked to hypertrophic and dilated cardiomyopathies, (2) genetic and stem cell based approaches to rescue hearts from failure imposed by myocardial infarction, ..
  29. Fo Motor Mechanisms that Power FoF1 ATP Synthesis
    Wayne D Frasch; Fiscal Year: 2013
    ..Some patients with cardiomyopathies or Parkinson's disease also have increased damage to these genes...
  30. Late Sodium Current Blockade in High-Risk ICD Patients - DCC
    Arthur J Moss; Fiscal Year: 2013
    ..Ischemia and cardiomyopathies are associated with a sodium overload of myocardial cells...
  31. The Role of Cardiomyopathic Troponin C Mutations in Skeletal and Cardiac Muscle C
    Jose Renato Pinto; Fiscal Year: 2013
    ..the primary Ca2+ sensor of striated muscle and has been a target of Hypertrophic (HCM) and Dilated (DCM) Cardiomyopathies. HCM or DCM are genetic disorders caused by the mutations in the TnC gene that are characterized by ..
  32. Regulation of Proteasome Function in Cardiomyopathies
    Sharlene M Day; Fiscal Year: 2013
    ..This proposal uniquely focuses on the role of the UPS in cardiomyopathies, progressive and often fatal heart muscle diseases...
  33. Conformations and interactions of insect olfactory receptor proteins
    ROBERT D RENTHAL; Fiscal Year: 2010
    ..Also, one component of the insect pheromone receptor is similar to human proteins involved in atherosclerosis, inflammatory response, metabolic syndrome, cardiomyopathies, high frequency hearing loss, and Gaucher's disease.
  34. Role of Nucleo-cytoskeleton Interactions in Cell Migration
    Gregg G Gundersen; Fiscal Year: 2013
    ..involved in nuclear positioning pathways cause a number of human diseases, including muscular dystrophies, cardiomyopathies, cerebellar ataxia and lissencephaly, these studies will also provide new avenues for understanding human ..
  35. Molecular Determinants of Arterial Remodeling in Atherogenesis
    Peter Libby; Fiscal Year: 2013
    ..cathepsins K and S, MMP-9 and -12, and neutrophil elastase, as well as from mice with genetically-induced cardiomyopathies. If we succeed in developing and validating novel biomarkers, we will apply them to banks of blood specimens ..
  36. The Role of Galectin-3 in Cardiac Remodeling and Heart Failure
    JENNIFER EN SIAN HO; Fiscal Year: 2013
    ..Carolyn Ho, an expert in noninvasive cardiovascular imaging and genetic cardiomyopathies. Dr...
  37. ROLE OF BETA-RECEPTOR SIGNALING IN CARDIOMYOPATHY
    Daniel Bernstein; Fiscal Year: 2010
    ..of the role of b-AR subtypes in cardiotoxic/cardioprotective signaling has therapeutic consequences for cardiomyopathies in general: if b1-ARs are toxic and b2-ARs are protective, as some have suggested, then future therapy of ..
  38. Role of Cardiac Troponin in Health and Disease
    JONATHAN PAUL DAVIS; Fiscal Year: 2013
    ..with ischemia-reperfusion injury and 3) are associated with familial hypertrophic, restrictive and dilated cardiomyopathies (HCM, RCM and DCM, respectively)...
  39. Determining the role of Fragile X proteins in the heart
    STEFANIE NOVAK; Fiscal Year: 2013
    ..by using extensive immunofluorescence microscopy and histology to analyze muscle defects and hallmarks of cardiomyopathies. Preliminary results indicate loss of dFMR1 significantly decreases heart rate and perturbs sarcomere ..
  40. INTEGRATED MECHANISMS OF CARDIAC MALADAPTATION
    R John Solaro; Fiscal Year: 2013
    ..findings in the projects to the diagnosis and to the development of novel therapies in familial and acquired cardiomyopathies and heart failure...
  41. Human Induced Pluripotent Cell Models of Pediatric Cardiac Disorders
    Ihor R Lemischka; Fiscal Year: 2013
    ..These studies could have important impact on the development of novel therapeutic strategies for these myocardial diseases, for which our current approaches are not curative. ..
  42. Novel pathways modulating Raf-mediated cardiac hypertrophy
    Matthew J Wolf; Fiscal Year: 2013
    ..Therefore, strategies using Drosophila as model to identify new genes that cause or modify cardiomyopathies were developed to circumvent some of the complexities associated with mammalian models of cardiovascular ..
  43. Cardiolipin and the mitochondrial ADP/ATP carrier interactome
    STEVEN MICHAEL CLAYPOOL; Fiscal Year: 2013
    ..are a multitude of pathologies caused by alterations in CL metabolism including both inherited and diabetic cardiomyopathies. Given the critical importance of CL for the Aac2p interactome, we hypothesize that the CL-dependent ADP/ATP ..
  44. Glycoconjugates and Cardiovascular Disease
    Gerald Warren Hart; Fiscal Year: 2013
    ..and intracellular glycoconjugates in the mechanisms protecting the heart or leading to atherosclerosis and cardiomyopathies, culminating in myocardial infarction and heart failure...
  45. Electro-mechanical Resynchronization Therapy For Arrhythmogenic Right Ventricular
    Harikrishna Tandri; Fiscal Year: 2012
    ..and clinical skills to achieve my long term objective to be a leader in diagnosis and management of genetic cardiomyopathies. The studies proposed in this grant will greatly expand our understanding of ARVC, and potential pave the way ..
  46. MYOSIN MOVEMENT IN VITRO-MOLECULAR CHARACTERIZATION
    JAMES ANTHONY SPUDICH; Fiscal Year: 2013
    ..In addition, inherited familial cardiomyopathies affecting 1 out of 500 people result from missense mutations in various cardiac muscle proteins, with ..
  47. Stem cells from the intervertebral disc: do they vary in degeneration?
    Zulma Gazit; Fiscal Year: 2010
    ..Yet it has been shown that during certain pathologies, such as osteoarthritis, osteoporosis and cardiomyopathies, abnormal growth pattern, and changes in differentiation of resident SCs are observed...
  48. Desmosomal Control of Cytoskeletal Organization
    Terry H Lechler; Fiscal Year: 2013
    ..desmosomes leads to a range of diseases in man including skin fragility and blistering disorders as well as cardiomyopathies. The textbook view of desmosomes is that they are rather passive structures that simply bind to the ..
  49. The Effects of Altered Contractility on Cardiac Myocyte Signaling and Hypertrophy
    MARIA LYNN VALENCIK; Fiscal Year: 2012
    ..and will help to establish new techniques for studying the molecular basis for the pathogenesis of cardiomyopathies. PUBLIC HEALTH RELEVANCE: In 2010 it is estimated that 81 million Americans have cardiovascular disease and ..
  50. Single Molecule Studies of Titin Elasticity
    Julio M Fernandez; Fiscal Year: 2013
    ..Indeed, genetic mutations of the titin gene were found to be a primary cause of dilated cardiomyopathies, one of the most common forms of heart disease...
  51. CTRIP: Genetic Testing to Individualize Management of Common Heart Diseases
    Gerald W Dorn; Fiscal Year: 2010
    ..Due in the majority of instances to dilated or ischemic cardiomyopathies, heart failure is a classic "complex disease", i.e...
  52. NMR Structural Analysis of Calcium Regulation in Muscle
    Gianluigi Veglia; Fiscal Year: 2013
    ..When SERCA2a functions outside this window, disruptions in Ca2+ homeostasis leads to dilated or hypertrophic cardiomyopathies, and ultimately heart failure...
  53. Inhibiting protein-primed replication of human enteroviruses.
    Catherine H Schein; Fiscal Year: 2013
    ..provided by applicant): Persistent infections with Human enteroviruses (HEV) are a major cause of dilated cardiomyopathies, and have been causally implicated in type 1 diabetes, gastrointestinal and nervous system disorders...
  54. iPSC-Derived Cardiomyocytes in Left Ventricular Non-Compaction Cardiomyopathy
    Daniel Bernstein; Fiscal Year: 2013
    ..LVNC has a unique phenotype that distinguishes it from dilated or hypertrophic cardiomyopathies: deep hypertrabeculation of the left ventricle...
  55. Iroquois Homeobox Transcription Factors in Heart Development and Physiology
    BENOIT GAETAN BRUNEAU; Fiscal Year: 2010
    ..likely to be at the root of several human congenital heart diseases that affect cardiac physiology, such as cardiomyopathies and arrhythmias...
  56. Rescue of Beta-Adrenergic Cardiomyopathy by Inhibition of Adenylyl Cyclase
    Dorothy E Vatner; Fiscal Year: 2012
    ..Hypothesis B: The rescue of the [unreadable]1-AR Tg or [unreadable]2-AR Tg cardiomyopathies by inhibition of AC5 permits enhanced exercise performance which is due to (1) improved cardiac output and ..
  57. Integrins and Caveolin Proteins in Cardiac Hypertrophy and Failure
    David M Roth; Fiscal Year: 2013
    ..in transducing mechanical signals, and that reduced expression and/ or mutation of ITGs and Cav3 can cause cardiomyopathies. How [unreadable]1 ITGs and Cav-3 functionally interact and by what mechanisms their coordinated responses ..
  58. Fibroblast Growth Factor Homologous Factors: Modulation of L-type Calcium Channel
    JESSICA AMENTA HENNESSEY; Fiscal Year: 2013
    ..by applicant): Background: A growing number of cardiovascular disorders such as arrhythmias and certain cardiomyopathies are attributable to ion channel dysfunction that results from mutations within channels...
  59. Oxidative Response Networks in Chagasic Cardiomyopathy
    Nisha Jain Garg; Fiscal Year: 2013
    ..the oxidative stress response pathways and networks that enhance the susceptibility to or progression of chagasic (and other) cardiomyopathies. We anticipate identifying novel targets for prevention and treatment of Chagas disease.
  60. Structure/Function Studies of Small Heat Shock Proteins
    Rachel E Klevit; Fiscal Year: 2013
    ..used to determine the structure of the most widely known ?B mutant, R120G ?B, involved in cataracts and cardiomyopathies (Aim 1)...
  61. Purkinje Cells and Arrhythmia Mechanisms
    Glenn I Fishman; Fiscal Year: 2013
    ..in the setting of acute ischemia, acquired heart disease or inherited syndromes including channelopathies and cardiomyopathies. The specialized cardiac conduction system (CCS) comprises a heterogeneous network of cells that orchestrate ..
  62. PATHOPHYSIOLOGY OF CARDIOMYOPATHY
    JUDITH GWATHMEY; Fiscal Year: 1991
    ..amount of indirect information has accumulated to suggest that intracellular Ca++ handling is abnormal in cardiomyopathies of various etiologies; the Ca++ indicator aequorin will be used to determine how changes in intracellular Ca++..
  63. ACAS 570 ANCHORED CELL ANALYZER AND SORTER
    RAYMOND HESTER; Fiscal Year: 1991
    ..of the poly(ADP-ribose) system in chemically-induced beta cell damage, (11) calcium transport in diabetic cardiomyopathies, (12) Na-Ca exchange in immature myocardium, (13) epidermal morphogenesis...
  64. CARDIOVASCULAR EFFECTS OF COCAINE
    MARK KNUEPFER; Fiscal Year: 2001
    ..we have identified variability in response characteristics that is related to variability in cocaine-induced cardiomyopathies but not to pressor responses...
  65. Local Signals and Macromolecular Architecture in Heart
    Terry Rogers; Fiscal Year: 2006
    ..intricate network has been recently emphasized by its links to acquired and inherited forms of human dilated cardiomyopathies. Despite the emerging appreciation for the crucial role of cardiac cytoskeletal networks, these structures, ..
  66. Chronic Stress or Psychostimulants on Central and Autonomic Nervous Systems
    Mark M Knuepfer; Fiscal Year: 2010
    ..Vascular responders are more susceptible to cocaine induced cardiomyopathies and hypertension compared to mixed responders...
  67. CYTOKINE-INDUCED ARRHYTHMIAS IN CONGESTIVE HEART FAILURE
    Barry London; Fiscal Year: 2004
    DESCRIPTION (the applicant's description verbatim): Arrhythmias are a major health problem in cardiomyopathies of both ischemic and non-ischemic origin...
  68. 2002 Gordon Research Conf. on Intermediate Filaments
    PIERRE COULOMBE; Fiscal Year: 2002
    ..g., skin, oral, and eye blistering diseases; inflammatory bowel diseases; liver disorders), muscle (e.g., cardiomyopathies; muscular dystrophy), neural tissue (e.g...
  69. Dynamic ECG Predictors of Sudden Cardiac Death in Ischemia Cardiomyopathy
    Mary Carey; Fiscal Year: 2007
    ..while expanding her expertise to analyze repolarization abnormalities in ambulatory patients with chronic cardiomyopathies. This K23 will allow the candidate, guided by her mentor John M. Canty Jr...
  70. Structual Analysis of Enterovirus Cell Entry Pathways
    JAMES HOGLE; Fiscal Year: 2002
    ..human diseases including: common colds, summer flu, poliomyelitis, encephalitis, meningitis, and a variety of cardiomyopathies. In previous funding periods several forms of poliovirus relevant to cell entry have been characterized by a ..
  71. Sympathetic Regulation of Endotoxemia in Drug Abuse
    MARK KNUEPFER; Fiscal Year: 2005
    ..In our model of cocaine-induced cardiomyopathies and hypertension in conscious rats, cocaine evokes substantial increases in systemic vascular resistance (..
  72. Structural Mechanisms Modulating Contraction in Normal and Diseased Heart
    Paul R Rosevear; Fiscal Year: 2010
    ..Altered phosphorylation can lead to specific disease states such as familial hypertrophic and dilated cardiomyopathies and end stage heart failure...