Glycobiology of α-dystroglycan and muscular dystrophy.
Most proteins are modified by glycans, which can modulate the biological properties and functions of glycoproteins. The major glycans can be classified into N-glycans and O-glycans according to their glycan-peptide linkage. This review will provide an overview of the O-mannosyl glycans, one subtype of O-glycans. Originally, O-mannosyl glycan was only known to be present on a limited number of glycoproteins, especially α-dystroglycan (α-DG). However, once a clear relationship was established between O-mannosyl glycan and the pathological mechanisms of some congenital muscular dystrophies in humans, research on the biochemistry and pathology of O-mannosyl glycans has been expanding. Because α-DG glycosylation is defective in congenital muscular dystrophies, which also feature abnormal neuronal migration, these disorders are collectively called α-dystroglycanopathies. In this article, I will describe the structure, biosynthesis and pathology of O-mannosyl glycans.
Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology, Tokyo 173-0015, Japan endo@tmig.or.jp.
Tamao
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Endo
D049030:Dystroglycans / Q000096:biosynthesis* / Q000378:metabolism; D054794:Glycomics; D006031:Glycosylation; D006801:Humans; D009136:Muscular Dystrophies / Q000235:genetics* / Q000378:metabolism / Q000473:pathology; D009154:Mutation; D011134:Polysaccharides / Q000096:biosynthesis*
D011134:Polysaccharides; D049030:Dystroglycans
O-mannosyl glycan; dystroglycan; glycan biosynthesis; glycosyltransferase; muscular dystrophy