Facilitated Cross-Bridge Interactions with Thin Filaments by Familial Hypertrophic Cardiomyopathy Mutations in α-Tropomyosin
Joint Authors
Compton, Lisa A.
Bienkiewicz, Ewa A.
Mihajlović, Goran
Wang, Fang
Miller, Victor F.
Chase, P. Bryant
Brunet, Nicolas M.
Asbury, Thomas M.
Grubich, Justin R.
Source
Journal of Biomedicine and Biotechnology
Issue
Vol. 2011, Issue 2011 (31 Dec. 2011), pp.1-12, 12 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2011-12-01
Country of Publication
Egypt
No. of Pages
12
Main Subjects
Abstract EN
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 structure and increased unordered structure.
When thin filaments were fully occupied by regulatory proteins, little or no motion was detected at pCa 9, and maximum speed (pCa 5) was similar for all tropomyosins.
Ca2+-responsiveness of filament sliding speed was increased either by increased pCa50 (V95A), reduced cooperativity n (D175N), or both (E180G).
When temperature was increased, thin filaments with E180G exhibited dysregulation at temperatures ~10°C lower, and much closer to body temperature, than WT.
When HMM density was reduced, thin filaments with D175N required fewer motors to initiate sliding or achieve maximum sliding speed.
American Psychological Association (APA)
Wang, Fang& Brunet, Nicolas M.& Grubich, Justin R.& Bienkiewicz, Ewa A.& Asbury, Thomas M.& Compton, Lisa A.…[et al.]. 2011. Facilitated Cross-Bridge Interactions with Thin Filaments by Familial Hypertrophic Cardiomyopathy Mutations in α-Tropomyosin. Journal of Biomedicine and Biotechnology،Vol. 2011, no. 2011, pp.1-12.
https://search.emarefa.net/detail/BIM-472015
Modern Language Association (MLA)
Wang, Fang…[et al.]. Facilitated Cross-Bridge Interactions with Thin Filaments by Familial Hypertrophic Cardiomyopathy Mutations in α-Tropomyosin. Journal of Biomedicine and Biotechnology No. 2011 (2011), pp.1-12.
https://search.emarefa.net/detail/BIM-472015
American Medical Association (AMA)
Wang, Fang& Brunet, Nicolas M.& Grubich, Justin R.& Bienkiewicz, Ewa A.& Asbury, Thomas M.& Compton, Lisa A.…[et al.]. Facilitated Cross-Bridge Interactions with Thin Filaments by Familial Hypertrophic Cardiomyopathy Mutations in α-Tropomyosin. Journal of Biomedicine and Biotechnology. 2011. Vol. 2011, no. 2011, pp.1-12.
https://search.emarefa.net/detail/BIM-472015
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-472015