Abstract
Background: A mutation found in titin has been linked to arrhythmogenic cardiomyopathy (AC). Results: The mutation increases Ig10 instability and susceptibility to degradation. Conclusion: The mutation compromises the local structure of Ig10 and has a significant effect on Ig10 unfolding dynamics. Significance: Titin is the first sarcomeric protein to be implicated in AC pathology; a novel titin-based disease mechanism is suggested.
Original language | English (US) |
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Pages (from-to) | 5303-5315 |
Number of pages | 13 |
Journal | Journal of Biological Chemistry |
Volume | 288 |
Issue number | 8 |
DOIs | |
State | Published - Feb 22 2013 |
ASJC Scopus subject areas
- Biochemistry
- Molecular Biology
- Cell Biology