Single molecule force spectroscopy on titin implicates immunoglobulin domain stability as a cardiac disease mechanism

Brian R. Anderson, Julius Bogomolovas, Siegfried Labeit, Henk Granzier

Research output: Contribution to journalArticle

25 Scopus citations

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 languageEnglish (US)
Pages (from-to)5303-5315
Number of pages13
JournalJournal of Biological Chemistry
Volume288
Issue number8
DOIs
StatePublished - Feb 22 2013

ASJC Scopus subject areas

  • Biochemistry
  • Molecular Biology
  • Cell Biology

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