Abstract:
: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 alpha dystroglycan. Alpha dystroglycan is an essential component of the dystrophin-glycoprotein complex, and aberrant glycosylation of alpha 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.
journal_name
Glycobiologyjournal_title
Glycobiologyauthors
Martin PT,Freeze HHdoi
10.1093/glycob/cwg077subject
Has Abstractpub_date
2003-08-01 00:00:00pages
67R-75Rissue
8eissn
0959-6658issn
1460-2423pii
cwg077journal_volume
13pub_type
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