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The Role of Alpha-Dystrobrevin in Striated Muscle
Department of Neurology, Osaka University Graduate School of Medicine, 2-2, D-4, Yamadaoka, Suita, Osaka 565-0871, Japan
Department of Neurology, University of Rochester Medical Center, 601 Elmwood Avenue, Box 645 URMC, Rochester, NY 14642, USA
* Author to whom correspondence should be addressed.
Received: 16 December 2010; in revised form: 29 January 2011 / Accepted: 23 February 2011 / Published: 4 March 2011
Abstract: Muscular dystrophies are a group of diseases that primarily affect striated muscle and are characterized by the progressive loss of muscle strength and integrity. Major forms of muscular dystrophies are caused by the abnormalities of the dystrophin glycoprotein complex (DGC) that plays crucial roles as a structural unit and scaffolds for signaling molecules at the sarcolemma. α-Dystrobrevin is a component of the DGC and directly associates with dystrophin. α-Dystrobrevin also binds to intermediate filaments as well as syntrophin, a modular adaptor protein thought to be involved in signaling. Although no muscular dystrophy has been associated within mutations of the α-dystrobrevin gene, emerging findings suggest potential significance of α-dystrobrevin in striated muscle. This review addresses the functional role of α-dystrobrevin in muscle as well as its possible implication for muscular dystrophy.
Keywords: dystrobrevin; syntrophin; dystrophin; DGC; muscular dystrophy; signaling; intermediate filament; splicing
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Nakamori, M.; Takahashi, M.P. The Role of Alpha-Dystrobrevin in Striated Muscle. Int. J. Mol. Sci. 2011, 12, 1660-1671.
Nakamori M, Takahashi MP. The Role of Alpha-Dystrobrevin in Striated Muscle. International Journal of Molecular Sciences. 2011; 12(3):1660-1671.
Nakamori, Masayuki; Takahashi, Masanori P. 2011. "The Role of Alpha-Dystrobrevin in Striated Muscle." Int. J. Mol. Sci. 12, no. 3: 1660-1671.