Hypertrophic Cardiomyopathy associated E22K Mutation in Myosin Regulatory Light Chain Decreases Calcium-Activated Tension and Stiffness and Reduces Myofilament Ca

Authors

Jiajia Zhang
Li Wang
Katarzyna Kazmierczak
Hang Yun
Danuta Szczesna-Cordary
Masataka Kawai

Publication Date

8-1-2021

Journal

The FEBS Journal

Abstract

UNLABELLED: We investigated the mechanisms associated with E22K mutation in myosin regulatory light chain (RLC), found to cause hypertrophic cardiomyopathy (HCM) in humans and mice. Specifically, we characterized the mechanical profiles of papillary muscle fibers from transgenic mice expressing human ventricular RLC wild-type (Tg-WT) or E22K mutation (Tg-E22K). Because the two mouse models expressed different amounts of transgene, the B6SJL mouse line (NTg) was used as an additional control. Mechanical experiments were carried out on Ca

SUB-DISCIPLINE: Bioenergetics.

DATABASE: The data that support the findings of this study are available from the corresponding authors upon reasonable request.

ANIMAL PROTOCOL: BK20150353 (Soochow University).

RESEARCH GOVERNANCE: School of Nursing: Hua-Gang Hu: seuboyh@163.com; Soochow University: Chen Ge chge@suda.edu.cn.

Keywords

Adenosine Triphosphate, Animals, Biomechanical Phenomena, Calcium, Cardiomyopathy, Hypertrophic, Elasticity, Female, Male, Mice, Mutation, Missense, Myocardial Contraction, Myofibrils, Myosin Light Chains