The hypertrophic cardiomyopathy myosin mutation R453C alters ATP binding and hydrolysis of human cardiac β-myosin

Abstract

Background: R453C is a mutation in human cardiac myosin and is associated with a high incidence of sudden cardiac death. Results: R453C alters few kinetic parameters, except for the conformational changes associated with ATP binding and hydrolysis. Conclusion: The closure of switch-2 on ATP is disrupted by R453C. Significance: This is the first detailed kinetic analysis of the motor domain of the human β-cardiac myosin carrying the R453C mutation. © 2014 by The American Society for Biochemistry and Molecular Biology, Inc.