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Dystroglycan in development and disease
Journal article   Peer reviewed

Dystroglycan in development and disease

Madeleine Durbeej, Michael D Henry and Kevin P Campbell
Current opinion in cell biology, Vol.10(5), pp.594-601
1998
DOI: 10.1016/S0955-0674(98)80034-3
PMID: 9818169

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Abstract

Our understanding of the structure and function of dystroglycan, a cell surface laminin/agrin receptor, has increased dramatically over the past two years. Structural studies, analysis of its binding partners, and targeted gene disruption have all contributed to the elucidation of the biological role of dystroglycan in development and disease. It is now apparent that dystroglycan plays a critical role in the pathogenesis of several muscular dystrophies and serves as a receptor for a human pathogen as well as being involved in early development, organ morphogenesis, and synaptogenesis.
Sia sialic acid FCMD Fukuyama-type congenital muscular dystrophy DGC dystrophin—glycoprotein complex Gal galactose OPL outer plexiform layer GlcNAc N-acetyl glucosamine AChR acetylcholine receptor Man mannose ECM extracellular matrix NMJ neuromuscular junction

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