Slower Calcium Handling Balances Faster Cross-Bridge Cycling in Human MYBPC3 HCM.

Josè Manuel PionerGiulia Vitale Sonette Steczina Marianna Langione Francesca MargaraLorenzo SantiniFrancesco GiardiniErica LazzeriNicoletta PiroddiBeatrice ScelliniChiara Palandri Maike SchuldtValentina SpinelliFrancesca Girolami Francesco Mazzarotto Jolanda van der Velden Elisabetta CerbaiChiara TesiIacopo Olivotto Alfonso Bueno-OrovioLeonardo SacconiRaffaele Coppini Corrado Poggesi Michael Regnier Corrado Poggesi

Published in: Circulation research (2023)

: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.

Keyphrases

hypertrophic cardiomyopathy
end stage renal disease
endothelial cells
newly diagnosed
high intensity
prognostic factors
left ventricular
heart failure
patient reported outcomes
smooth muscle
binding protein