Ontology highlight
ABSTRACT:
SUBMITTER: Praissman JL
PROVIDER: S-EPMC4924997 | biostudies-literature | 2016 Apr
REPOSITORIES: biostudies-literature
eLife 20160429
Multiple glycosyltransferases are essential for the proper modification of alpha-dystroglycan, as mutations in the encoding genes cause congenital/limb-girdle muscular dystrophies. Here we elucidate further the structure of an O-mannose-initiated glycan on alpha-dystroglycan that is required to generate its extracellular matrix-binding polysaccharide. This functional glycan contains a novel ribitol structure that links a phosphotrisaccharide to xylose. ISPD is a CDP-ribitol (ribose) pyrophosphor ...[more]