期刊
JOURNAL OF BIOCHEMISTRY
卷 157, 期 1, 页码 1-12出版社
OXFORD UNIV PRESS
DOI: 10.1093/jb/mvu066
关键词
dystroglycan; glycan biosynthesis; glycosyltransferase; muscular dystrophy; O-mannosyl glycan
资金
- NCNP [26-8]
- Grants-in-Aid for Scientific Research [25293016] Funding Source: KAKEN
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 alpha-dystroglycan (alpha-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 alpha-DG glycosylation is defective in congenital muscular dystrophies, which also feature abnormal neuronal migration, these disorders are collectively called alpha-dystroglycanopathies. In this article, I will describe the structure, biosynthesis and pathology of O-mannosyl glycans.
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