Hypertrophic cardiomyopathy:a paradigm for myocardial energy depletion.

  title={Hypertrophic cardiomyopathy:a paradigm for myocardial energy depletion.},
  author={Houman Ashrafian and Charles S. Redwood and Edward Blair and H. V. Watkins},
  journal={Trends in genetics : TIG},
  volume={19 5},
Genetic analysis of hypertrophic cardiomyopathy (HCM), a mendelian form of cardiac hypertrophy, indicates that the primary defect is in sarcomeric function. However, the initial proposal that depressed myocardial contraction leads to a 'compensatory' hypertrophy has proven inconsistent with laboratory and clinical evidence. Drawing on observations of mutant contractile protein function, together with mouse models and clinical studies, we propose that sarcomeric HCM mutations lead to inefficient… CONTINUE READING


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