Destabilization of the dystrophin-glycoprotein complex without functional deficits in α-dystrobrevin null muscle

Tina M. Bunnell, Michel A. Jaeger, Daniel P. Fitzsimons, Kurt W. Prins, James M. Ervasti

Research output: Contribution to journalArticle

14 Scopus citations

Abstract

α-Dystrobrevin is a component of the dystrophin-glycoprotein complex (DGC) and is thought to have both structural and signaling roles in skeletal muscle. Mice deficient for α-dystrobrevin (adbn-/-) exhibit extensive myofiber degeneration and neuromuscular junction abnormalities. However, the biochemical stability of the DGC and the functional performance of adbn-/- muscle have not been characterized. Here we show that the biochemical association between dystrophin and β-dystroglycan is compromised in adbn-/- skeletal muscle, suggesting that α-dystrobrevin plays a structural role in stabilizing the DGC. However, despite muscle cell death and DGC destabilization, costamere organization and physiological performance is normal in adbn-/- skeletal muscle. Our results demonstrate that myofiber degeneration alone does not cause functional deficits and suggests that more complex pathological factors contribute to the development of muscle weakness in muscular dystrophy.

Original languageEnglish (US)
Article numbere2604
JournalPloS one
Volume3
Issue number7
DOIs
StatePublished - Jul 2 2008

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