Reaction: POMGNT1 transfers GlcNAc from UDP-GlcNAc to Man-O-Ser-DAG1
- in pathway: O-linked glycosylation
Golgi membrane resident protein O-linked-mannose beta-1,2-N-acetylglucosaminyltransferase 1 (POMGNT1; MIM:606822) mediates the transfer of N-acetylglucosaminyl (GlcNAc) residues to mannosylated proteins in the Golgi lumen. It can transfer GlcNAc to mannose-O-serine-dystroglycan (Man-DAG1) with a beta-1,2 linkage. Defects in POMGNT1 result in disrupted glycosylation of DAG1 and can cause congenital muscular dystrophy-dystroglycanopathies of varying severity (Yoshida et al. 2001).
Reaction - small molecule participants:
UDP [Golgi lumen]
UDP-GlcNAc [Golgi lumen]
Reactome.org reaction link: R-HSA-5617037
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Reaction input - small molecules:
UDP-N-acetyl-alpha-D-glucosamine(2-)
Reaction output - small molecules:
UDP(3-)
Reactome.org link: R-HSA-5617037