Hypertrophic cardiomyopathy is an autosomal-dominant disease.Disease-causing mutations have been found in genes encoding structural components of the thick and thin filament systems of cardiac myocyte;it has therefore...Hypertrophic cardiomyopathy is an autosomal-dominant disease.Disease-causing mutations have been found in genes encoding structural components of the thick and thin filament systems of cardiac myocyte;it has therefore been named as a disease of sarcomere.Many approaches have been used to characterize the pathogenesis of the desease.Transgenic animal models have been created to gain further insight into the pathogenesis of this disease.Most of these models has been made in mice;however,recently a transgenic rabbit model has been created.In addition,there are several natural-occurring forms of HCM in animals.The discovery of responsible genes and the elucidation of the molecular mechanisms of pathogenesis through the use of animal models promise improved and early diagnosis and the potential for mechanism-based therapeutics.展开更多
文摘Hypertrophic cardiomyopathy is an autosomal-dominant disease.Disease-causing mutations have been found in genes encoding structural components of the thick and thin filament systems of cardiac myocyte;it has therefore been named as a disease of sarcomere.Many approaches have been used to characterize the pathogenesis of the desease.Transgenic animal models have been created to gain further insight into the pathogenesis of this disease.Most of these models has been made in mice;however,recently a transgenic rabbit model has been created.In addition,there are several natural-occurring forms of HCM in animals.The discovery of responsible genes and the elucidation of the molecular mechanisms of pathogenesis through the use of animal models promise improved and early diagnosis and the potential for mechanism-based therapeutics.
