The Role of Alpha-Dystrobrevin in Striated Muscle

International Journal of Molecular Sciences

关键词: dystrobrevin;    syntrophin;    dystrophin;    DGC;    muscular dystrophy;    signaling;    intermediate filament;    splicing;   

DOI  :  10.3390/ijms12031660

来源: DOAJ

【 摘 要 】

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.

【 授权许可】

Unknown