Article
Facilitated cross-bridge interactions with thin filaments by familial hypertrophic cardiomyopathy mutations in α-tropomyosin.
Journal of biomedicine & biotechnology - 1 Jan 2011
Wang Fang, Brunet Nicolas M, Grubich Justin R, Bienkiewicz Ewa A, Asbury Thomas M, Compton Lisa A, Mihajlović Goran, Miller Victor F, Chase P Bryant
Abstract excerpt
Familial hypertrophic cardiomyopathy (FHC) is a disease of cardiac sarcomeres. To identify molecular mechanisms underlying FHC pathology, functional and structural differences in three FHC-related mutations in recombinant α-Tm (V95A, D175N, and E180G) were characterized using both conventional and modified in vitro motility assays and circular dichroism spectroscopy. Mutant Tm's exhibited reduced α-helical...
Topics
- Actins
- Animals
- Calcium
- Cardiomyopathy, Hypertrophic, Familial
- Electrophoresis, Polyacrylamide Gel
- Humans
- Male
- Mutation
- Myosin Subfragments
- Rabbits
- Temperature
- Tropomyosin