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Journal of Biomedicine and Biotechnology
Volume 2010 (2010), Article ID 740403, 17 pages
http://dx.doi.org/10.1155/2010/740403
Review Article

Biological Role of Dystroglycan in Schwann Cell Function and Its Implications in Peripheral Nervous System Diseases

1Centre for Neuroregeneration, University of Edinburgh, Chancellor's Building, 49 Little France Crescent, Edinburgh EH16 4SB, UK
2Faculty of Medical Sciences, Teikyo University of Science, 2-2 Senju-Sakuragi, Adachi-ku, Tokyo 120-0045, Japan
3Department of Neurology and Neuroscience, Teikyo University, 2-11-1 Kaga, Itabashi-ku, Tokyo 173-8605, Japan

Received 22 October 2009; Accepted 20 April 2010

Academic Editor: Chung-Liang Chien

Copyright © 2010 Toshihiro Masaki and Kiichiro Matsumura. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Dystroglycan is a central component of the dystrophin-glycoprotein complex (DGC) that links extracellular matrix with cytoskeleton, expressed in a variety of fetal and adult tissues. Dystroglycan plays diverse roles in development and homeostasis including basement membrane formation, epithelial morphogenesis, membrane stability, cell polarization, and cell migration. In this paper, we will focus on biological role of dystroglycan in Schwann cell function, especially myelination. First, we review the molecular architecture of DGC in Schwann cell abaxonal membrane. Then, we will review the loss-of-function studies using targeted mutagenesis, which have revealed biological functions of each component of DGC in Schwann cells. Based on these findings, roles of dystroglycan in Schwann cell function, in myelination in particular, and its implications in diseases will be discussed in detail. Finally, in view of the fact that understanding the role of dystroglycan in Schwann cells is just beginning, future perspectives will be discussed.