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O-Mannosyl Glycan and Muscular Dystrophy

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Abstract

Glycosylation is an important posttranslational modification in mammals. The major glycans of glycoproteins can be classified into two groups, N-glycans and O-glycans, according to their glycan-peptide linkage regions. The development of sensitive methods for analyses of glycan structures has identified O-mannosyl glycans in mammals; these structures used to be considered specific to yeast. Originally, O-mannosyl glycan was considered to be present on a limited number of glycoproteins of the brain, nerves, and skeletal muscles, especially on α-dystroglycan (α-DG). However, since a clear relationship between O-mannosyl glycan and the pathomechanisms of some congenital muscular dystrophies in humans was established, this glyco field has been expanding both biochemically and pathologically. Because the glycosylation of α-DG is defective in congenital muscular dystrophies that show muscular dystrophy with abnormal neuronal migration, these disorders are collectively called α-dystroglycanopathy. Although it is known that O-mannosyl glycans have various structures, the biosynthetic pathway responsible remains only partially understood. In addition, many new causative genes of α-dystroglycanopathies are continuously being found. In this article, we discuss the structure, biosynthesis, and pathology of O-mannosyl glycans.

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  1. Molecular Glycobiology, Research Team for Mechanism of Aging, Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology, Itabashi-ku, Tokyo, 173-0015, Japan

    Hiroshi Manya & Tamao Endo

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  1. Hiroshi Manya
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  2. Tamao Endo
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  1. Glycometabolome Team, Systems Glycobiology Research Group, RIKEN-Max-Planck Joint Research Center for Systems Chemical Biology, RIKEN Global Research Cluster, Wako, Saitama, Japan

    Tadashi Suzuki

  2. Department of Analytical Biochemistry, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan

    Kazuaki Ohtsubo

  3. Disease Glycomics Team, Systems Glycobiology Research Group, RIKEN-Max-Planck Joint Research Center for Systems Chemical Biology, RIKEN Global Research Cluster, Wako, Saitama, Japan

    Naoyuki Taniguchi

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Manya, H., Endo, T. (2015). O-Mannosyl Glycan and Muscular Dystrophy. In: Suzuki, T., Ohtsubo, K., Taniguchi, N. (eds) Sugar Chains. Springer, Tokyo. https://doi.org/10.1007/978-4-431-55381-6_15

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