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A Contraction Stress Model of Hypertrophic Cardiomyopathy due to Sarcomere Mutations.

Abstract：

:Thick-filament sarcomere mutations are a common cause of hypertrophic cardiomyopathy (HCM), a disorder of heart muscle thickening associated with sudden cardiac death and heart failure, with unclear mechanisms. We engineered four isogenic induced pluripotent stem cell (iPSC) models of β-myosin heavy chain and myosin-binding protein C3 mutations, and studied iPSC-derived cardiomyocytes in cardiac microtissue assays that resemble cardiac architecture and biomechanics. All HCM mutations resulted in hypercontractility with prolonged relaxation kinetics in proportion to mutation pathogenicity, but not changes in calcium handling. RNA sequencing and expression studies of HCM models identified p53 activation, oxidative stress, and cytotoxicity induced by metabolic stress that can be reversed by p53 genetic ablation. Our findings implicate hypercontractility as a direct consequence of thick-filament mutations, irrespective of mutation localization, and the p53 pathway as a molecular marker of contraction stress and candidate therapeutic target for HCM patients.

journal_name

Stem Cell Reports

journal_title

Stem cell reports

authors

Cohn R,Thakar K,Lowe A,Ladha FA,Pettinato AM,Romano R,Meredith E,Chen YS,Atamanuk K,Huey BD,Hinson JT

doi

10.1016/j.stemcr.2018.11.015

subject

Has Abstract

pub_date

2019-01-08 00:00:00

pages

71-83

issue

1

issn

2213-6711

pii

S2213-6711(18)30483-1

journal_volume

12

pub_type

杂志文章

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