Journal
JOURNAL OF EXPERIMENTAL BIOLOGY
Volume 219, Issue 2, Pages 161-167Publisher
COMPANY OF BIOLOGISTS LTD
DOI: 10.1242/jeb.125930
Keywords
Cardiac myosin; Cardiomyopathy mutations; Force-velocity curves
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Funding
- National Institutes of Health [GM33289, HL117138]
- Human Frontiers Science Program
- National Institutes of Health Cellular, Biochemical, and Molecular Sciences Training Grant
- Stanford Interdisciplinary Graduate Fellowship
- Stanford Dean's Postdoctoral Fellowship
- National Science Foundation Graduate Research Fellowship Program
- Lucile Packard Child Health Research Institute Postdoctoral Grant
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Hypertrophic cardiomyopathy is the most frequently occurring inherited cardiovascular disease, with a prevalence of more than one in 500 individuals worldwide. Genetically acquired dilated cardiomyopathy is a related disease that is less prevalent. Both are caused by mutations in the genes encoding the fundamental force-generating protein machinery of the cardiac muscle sarcomere, including human beta-cardiac myosin, the motor protein that powers ventricular contraction. Despite numerous studies, most performed with non-human or non-cardiac myosin, there is no clear consensus about the mechanism of action of these mutations on the function of human beta-cardiac myosin. We are using a recombinantly expressed human beta-cardiac myosin motor domain along with conventional and new methodologies to characterize the forces and velocities of the mutant myosins compared with wild type. Our studies are extending beyond myosin interactions with pure actin filaments to include the interaction of myosin with regulated actin filaments containing tropomyosin and troponin, the roles of regulatory light chain phosphorylation on the functions of the system, and the possible roles of myosin binding protein-C and titin, important regulatory components of both cardiac and skeletal muscles.
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