Key result
Truncated cMyBP-C mutants M6t and M7t showed markedly lower protein expression than wild type (70±4% and 11±5% of WT, p<0.05) due to preferential degradation by the ubiquitin-proteasome system.
Population
Neonatal rat cardiomyocytes
Comparison
Adenovirus-mediated expression of myc-tagged… vs Wild type (WT) cMyBP-C
Design
Preclinical
Authors
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Implicates UPS degradation in FHC pathogenesis from animal data; leaves open clinical relevance and therapeutic potential.
p-value: p=<0.05
Truncated cMyBP-C mutants associated with familial hypertrophic cardiomyopathy are preferentially degraded by the ubiquitin-proteasome system, which impairs its proteolytic capacity and may contribute to FHC pathogenesis.
Sarikas et al. (2005) studied Familial hypertrophic cardiomyopathy (FHC). Truncated cMyBP-C mutants (M6t, M7t) vs. Wild type (WT) cMyBP-C was evaluated on Protein expression levels (p=<0.05). Truncated cMyBP-C mutants M6t and M7t showed markedly lower protein expression than wild type (70±4% and 11±5% of WT, p<0.05) due to preferential degradation by the ubiquitin-proteasome system.
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