Myosin Regulatory Light Chain Phosphorylation Rescues Cardiac Dysfunction Caused by Familial Hypertrophic Cardiomyopathy-Linked Mutations

Abstract

In this report, we compared the role of cardiac myosin regulatory light chain (RLC) phosphorylation on cardiac function in skinned muscle preparations containing two RLC mutations, R58Q (arginine to glutamine) and D166V (aspartic acid to valine), both linked with a malignant disease phenotype. Previous studies on D166V-transgenic mice showed that the myosin light chain kinase (MLCK)-induced phosphorylation of D166V mouse myocardium was able to alleviate detrimental functional effects caused by this mutation.