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Cardiac myosin binding protein C insufficiency leads to early onset of mechanical dysfunction

Candida L Desjardins1, Yong Chen, Arthur T Coulton

  • 1Department of Physiology and Biophysics, Case Western Reserve University, Cleveland, OH 44106, USA.

Circulation. Cardiovascular Imaging
|December 14, 2011
PubMed

Insights

Decreased cardiac myosin binding protein C (cMyBPC) expression in mice leads to early cardiac dysfunction. These findings suggest a link between reduced cMyBPC and hypertrophic cardiomyopathy (HCM) development.

Area of Science:

  • Cardiovascular Biology
  • Molecular Cardiology
  • Cardiac Mechanics

Background:

  • Genetic mutations leading to decreased cardiac myosin binding protein C (cMyBPC) expression are implicated in hypertrophic cardiomyopathy (HCM).
  • The precise mechanisms linking cMyBPC levels to contractile dysfunction in HCM remain incompletely understood.

Purpose of the Study:

  • To investigate the impact of reduced cMyBPC expression on cardiac mechanical function in vivo and in vitro.
  • To elucidate the early functional consequences of cMyBPC deficiency in the context of potential HCM development.

Main Methods:

  • Evaluation of cardiac mechanical function in young cMyBPC knockout (cMyBPC(-/-)) and heterozygous (cMyBPC(±)) mice compared to wild-type (WT) controls.
  • In vitro assessment of skinned myocardium to analyze cross-bridge kinetics and stretch activation.
  • In vivo assessment using cardiac MRI to measure left ventricular (LV) strain, strain rates, and torsion.

Main Results:

  • cMyBPC(-/-) hearts showed accelerated cross-bridge kinetics and severely depressed LV strain and torsion in vivo.
  • cMyBPC(±) hearts had 23±5% less cMyBPC, with subtle accelerations in cross-bridge recruitment and reduced LV torsion and circumferential strain rates.
  • No overt hypertrophy was observed in cMyBPC(±) mice, despite functional deficits.

Conclusions:

  • Even modest reductions in cMyBPC expression can cause early-onset, subtle alterations in cardiac cross-bridge kinetics and LV mechanical function.
  • These early functional changes associated with decreased cMyBPC may represent a preclinical stage contributing to HCM development.
Abstract

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