Glycobiology Advance Access originally published online on May 7, 2003
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Glycobiology, 2003, Vol. 13, No. 8 67R-75R
© 2003 Oxford University Press
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Glycobiology of neuromuscular disorders
2 Department of Neuroscience, Glycobiology Research and Training Center, University of California, San Diego, School of Medicine, La Jolla, CA 92093-0691
3 Glycobiology and Carbohydrate Chemistry Program, Burnham Institute, 10901 North Torrey Pines Road, La Jolla, CA 92037
accepted on April 24, 2003
There has been a recent explosion in the identification of neuromuscular diseases caused by mutations in genes that affect carbohydrate metabolism or protein glycosylation. A number of these findings relate to defects in the glycosylation of 
dystroglycan. Dystroglycan is an essential component of the dystrophin–glycoprotein complex, and aberrant glycosylation of dystroglycan is associated with multiple forms of muscular dystrophy in mice and humans. We review the evidence that defects in dystroglycan glycosylation cause muscular dystrophy. In addition, we review evidence that glycobiology is important in other disorders that affect muscle, including hereditary inclusion body myopathy type II and congenital disorders of glycosylation. Finally, we discuss the long-term potential of glycotherapies for muscle disorders.
1 To whom correspondence should be addressed; e-mail: pmartin{at}ucsd.edu
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