Dystroglycan: from biosynthesis to pathogenesis of human disease

被引:435
作者
Barresi, R
Campbell, KP [1 ]
机构
[1] Univ Iowa, Howard Hughes Med Inst, Dept Physiol & Biophys, Roy J & Lucille A Carver Coll Med, Iowa City, IA 52242 USA
[2] Univ Iowa, Howard Hughes Med Inst, Dept Neurol, Roy J & Lucille A Carver Coll Med, Iowa City, IA 52242 USA
[3] Univ Iowa, Howard Hughes Med Inst, Dept Internal Med, Roy J & Lucille A Carver Coll Med, Iowa City, IA 52242 USA
关键词
dystroglycan; extracellular matrix; glycosylation;
D O I
10.1242/jcs.02814
中图分类号
Q2 [细胞生物学];
学科分类号
071009 ; 090102 ;
摘要
alpha- and beta-dystroglycan constitute a membrane-spanning complex that connects the extracellular matrix to the cytoskeleton. Although a structural role for dystroglycan had been identified, biochemical and genetic discoveries have recently highlighted the significance of post-translational processing for dystroglycan function. Glycosylation is the crucial modification that modulates the function of dystroglycan as a receptor for extracellular binding partners. It has become clear that perturbation of dystroglycan glycosylation is the central event in the pathogenesis of several complex disorders, and recent advances suggest that glycosylation could be modulated to ameliorate the pathological features. Our increased understanding of the mechanisms of interaction of dystroglycan with its ligands has become an essential tool in deciphering the biological processes related to the human diseases in which the proteins are implicated.
引用
收藏
页码:199 / 207
页数:9
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