Slower Calcium Handling Balances Faster Cross-Bridge Cycling in Human <i>MYBPC3</i> HCM

Abstract

HCM-related <i>MYBPC3</i>:c772G>A mutation invariably impairs sarcomere energetics and cross-bridge cycling. Compensatory electrophysiological changes (eg, reduced potassium channel expression) appear to preserve twitch contraction parameters, but may expose patients to greater arrhythmic propensity and disease progression. Therapeutic approaches correcting the primary sarcomeric defects may prevent secondary cardiomyocyte remodeling.