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- Title
Atrogin-1 and MuRF1 regulate cardiac MyBP-C levels via different mechanisms.
- Authors
Mearini, Giulia; Gedicke, Christina; Schlossarek, Saskia; Witt, Christian C.; Krämer, Elisabeth; Cao, Peirang; Gomes, Marcelo D.; Lecker, Stewart H.; Labeit, Siegfried; Willis, Monte S.; Eschenhagen, Thomas; Carrier, Lucie
- Abstract
Aims: Familial hypertrophic cardiomyopathy (FHC) is frequently caused by cardiac myosin-binding protein C (cMyBP-C) gene mutations, which should result in C-terminal truncated mutants. However, truncated mutants were not detected in myocardial tissue of FHC patients and were rapidly degraded by the ubiquitin-proteasome system (UPS) after gene transfer in cardiac myocytes. Since the diversity and specificity of UPS regulation lie in E3 ubiquitin ligases, we investigated whether the muscle-specific E3 ligases atrogin-1 or muscle ring finger protein-1 (MuRF1) mediate degradation of truncated cMyBP-C.
- Subjects
UBIQUITIN; LIGASES; HYPERTROPHIC cardiomyopathy; MYOSIN; CARRIER proteins; CARDIOMYOPATHIES; GENETIC mutation
- Publication
Cardiovascular Research, 2010, Vol 85, Issue 2, p357
- ISSN
0008-6363
- Publication type
Article
- DOI
10.1093/cvr/cvp348