Torn apart: membrane rupture in muscular dystrophies and associated cardiomyopathies.
Muscular dystrophies are often caused by mutations in cytoskeletal proteins that render cells more susceptible to strain-induced injury in mechanically active tissues such as skeletal or cardiac muscle. In this issue of the JCI, Han et al. report that dysferlin participates in membrane resealing in cardiomyocytes and that exercise results in increased membrane damage and disturbed cardiac function in dysferlin-deficient mice (see the related article beginning on page 1805). Thus, in addition to repetitive membrane damage, inadequate membrane repair may participate in the pathogenesis of muscular dystrophies and cardiomyopathies.
Lammerding
Jan
J
Cardiovascular Division, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA 02139, USA.
Lee
Richard T
RT
D000818:Animals; D009202:Cardiomyopathies / Q000378:metabolism* / Q000473:pathology* / Q000628:therapy; D000073939:Dysferlin; D006801:Humans; D008565:Membrane Proteins / Q000378:metabolism; D008566:Membranes / Q000378:metabolism / Q000473:pathology
C497505:Dysf protein, mouse; D000073939:Dysferlin; D008565:Membrane Proteins