Int. J. Mol. Sci. 2011, 12(3), 1660-1671; doi:10.3390/ijms12031660

The Role of Alpha-Dystrobrevin in Striated Muscle

 and 1,*
Received: 16 December 2010; in revised form: 29 January 2011 / Accepted: 23 February 2011 / Published: 4 March 2011
(This article belongs to the Special Issue Advances in Muscle Contraction Studies)
View Full-Text   |   Download PDF [329 KB, uploaded 19 June 2014]
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
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Export to BibTeX |

MDPI and ACS Style

Nakamori, M.; Takahashi, M.P. The Role of Alpha-Dystrobrevin in Striated Muscle. Int. J. Mol. Sci. 2011, 12, 1660-1671.

AMA Style

Nakamori M, Takahashi MP. The Role of Alpha-Dystrobrevin in Striated Muscle. International Journal of Molecular Sciences. 2011; 12(3):1660-1671.

Chicago/Turabian Style

Nakamori, Masayuki; Takahashi, Masanori P. 2011. "The Role of Alpha-Dystrobrevin in Striated Muscle." Int. J. Mol. Sci. 12, no. 3: 1660-1671.

Int. J. Mol. Sci. EISSN 1422-0067 Published by MDPI AG, Basel, Switzerland RSS E-Mail Table of Contents Alert