title: Cardiac phenotype of Duchenne Muscular Dystrophy: insights from cellular studies.
abstract: Dilated cardiomyopathy is a serious and almost inevitable complication of Duchenne Muscular Dystrophy, a devastating and fatal disease of skeletal muscle resulting from the lack of functional dystrophin, a protein linking the cytoskeleton to the extracellular matrix. Ultimately, it leads to congestive heart failure and arrhythmias resulting from both cardiac muscle fibrosis and impaired function of the remaining cardiomyocytes. Here we summarize findings obtained in several laboratories, focusing on cellular mechanisms that result in degradation of cardiac functions in dystrophy.
authors.affiliation: Department of Pharmacology and Physiology, University of Medicine and Dentistry - NJMS, Newark, NJ 07103, USA. nshiroko@umdnj.edu
authors.forename: Natalia
authors.initials: N
authors.lastname: Shirokova
authors.forename: Ernst
authors.initials: E
authors.lastname: Niggli
mesh_terms: D001145:Arrhythmias, Cardiac / Q000150:complications / Q000378:metabolism / Q000473:pathology; D002311:Cardiomyopathy, Dilated / Q000150:complications / Q000235:genetics* / Q000503:physiopathology; D016189:Dystrophin / Q000172:deficiency / Q000235:genetics* / Q000378:metabolism; D006333:Heart Failure / Q000150:complications / Q000378:metabolism / Q000473:pathology; D006801:Humans; D018482:Muscle, Skeletal / Q000378:metabolism / Q000503:physiopathology; D020388:Muscular Dystrophy, Duchenne / Q000150:complications / Q000235:genetics* / Q000503:physiopathology; D032383:Myocytes, Cardiac / Q000378:metabolism / Q000473:pathology*
chemical_list: D016189:Dystrophin