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Intermediate filaments and the function of the dystrophin-protein complex

Blake, Derek J. and Martin-Rendon, Enca 2002. Intermediate filaments and the function of the dystrophin-protein complex. Trends in Cardiovascular Medicine 12 (5) , pp. 224-228. 10.1016/S1050-1738(02)00166-4

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Intermediate filament (IF) proteins and the dystrophin-associated protein complex (DPC) play important roles in cardiac and skeletal muscle. Both systems are mutated in several different forms of inherited muscular dystrophy and cardiomyopathy. Recently two articles have been published that propose a physical link between the DPC and the IF network in muscle. Two novel IF proteins, syncoilin and desmuslin, have been identified as binding partners for the dystrophin-associated protein, α-dystrobrevin, in muscle. These novel interactions suggest that α-dystrobrevin may tether the IF protein network to the DPC. Mice lacking α-dystrobrevin develop muscular dystrophy without perturbing the assembly of the DPC at the muscle membrane, suggesting the involvement of other non-DPC proteins in the disease. The interaction between the DPC and the IF network may be disrupted in patients with Duchenne muscular dystrophy and in mice lacking α-dystrobrevin.

Item Type: Article
Date Type: Publication
Status: Published
Schools: Medicine
MRC Centre for Neuropsychiatric Genetics and Genomics (CNGG)
Subjects: R Medicine > R Medicine (General)
Publisher: Cell Press
ISSN: 1050-1738
Last Modified: 04 Jun 2017 05:10

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