Book chapter
Dystroglycan: Extracellular Matrix Receptor that Links to Cytoskeleton
Glycoscience: Biology and Medicine, pp.1245-1251
Springer Japan
10/20/2014
DOI: 10.1007/978-4-431-54841-6_173
Abstract
Dystroglycan is a highly glycosylated extracellular matrix receptor that is linked to the cytoskeleton and expressed in many tissues. It plays a crucial role in a variety of biological processes, including maintenance of the skeletal muscle membrane integrity and development of the central nervous system. Dystroglycanopathies are muscular dystrophies in which aberrant posttranslational modification results in a reduction in the number of [xylose-glucuronic acid] repeats on the α-dystroglycan subunit. This repeat is essential for binding between α-dystroglycan and its laminin-G domain-containing ligands within the extracellular matrix (i.e., laminin, perlecan, and agrin). Recent genetic and biochemical data have shown that mutations in at least 17 genes that encode enzymes implicated in posttranslational processing (including known and putative glycosyltransferases and a novel mannose kinase) lead to such reductions and cause congenital or limb-girdle muscular dystrophies that are sometimes accompanied by brain and eye abnormalities. In this chapter, we review the history of dystroglycan research, as well as our current understanding of the molecular underpinnings of dystroglycan function, in particular its posttranslational modifications.
Details
- Title: Subtitle
- Dystroglycan: Extracellular Matrix Receptor that Links to Cytoskeleton
- Creators
- Yuji Hara - University of Iowa, Molecular Physiology and BiophysicsKevin P Campbell - University of Iowa, Molecular Physiology and Biophysics
- Resource Type
- Book chapter
- Publication Details
- Glycoscience: Biology and Medicine, pp.1245-1251
- DOI
- 10.1007/978-4-431-54841-6_173
- Publisher
- Springer Japan; Tokyo
- Language
- English
- Date published
- 10/20/2014
- Academic Unit
- Neurology; Molecular Physiology and Biophysics; Iowa Neuroscience Institute
- Record Identifier
- 9984025081502771
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