Article
Impairment of the ubiquitin-proteasome system by truncated cardiac myosin binding protein C mutants.
Cardiovascular research - 1 Apr 2005
Sarikas Antonio, Carrier Lucie, Schenke Carolus, Doll Daniela, Flavigny Jeanne, Lindenberg Katrin S, Eschenhagen Thomas, Zolk Oliver
Abstract excerpt
OBJECTIVE: Most cardiac myosin binding protein C (cMyBP-C) gene mutations causing familial hypertrophic cardiomyopathy (FHC) result in C-terminal truncated proteins. However, truncated cMyBP-Cs were undetectable in myocardial tissue of FHC patients. In the present study, we investigated whether truncated cMyBP-Cs are subject to accelerated degradation by the lysosome or ubiquitin-proteasome system (UPS). METHODS...
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