Three perspectives on the molecular basis of hypercontractility caused by hypertrophic cardiomyopathy mutations

Pflugers Arch. 2019 May;471(5):701-717. doi: 10.1007/s00424-019-02259-2. Epub 2019 Feb 15.

Abstract

Several lines of evidence suggest that the primary effect of hypertrophic cardiomyopathy mutations in human β-cardiac myosin is hypercontractility of the heart, which leads to subsequent hypertrophy, fibrosis, and myofilament disarray. Here, I describe three perspectives on the molecular basis of this hypercontractility. The first is that hypercontractility results from changes in the fundamental parameters of the actin-activated β-cardiac myosin chemo-mechanical ATPase cycle. The second considers that hypercontractility results from an increase in the number of functionally accessible heads in the sarcomere for interaction with actin. The final and third perspective is that load dependence of contractility is affected by cardiomyopathy mutations and small-molecule effectors in a manner that changes the power output of cardiac contraction. Experimental approaches associated with each perspective are described along with concepts of therapeutic approaches that could prove valuable in treating hypertrophic cardiomyopathy.

Keywords: Hypertrophic cardiomyopathy; Interacting heads motif; Load; Super-relaxed state; β-Cardiac myosin.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Animals
  • Cardiomyopathy, Hypertrophic / genetics*
  • Cardiomyopathy, Hypertrophic / metabolism
  • Cardiomyopathy, Hypertrophic / physiopathology
  • Humans
  • Mutation
  • Myocardial Contraction*
  • Ventricular Myosins / genetics*
  • Ventricular Myosins / metabolism

Substances

  • Ventricular Myosins