Reaction: GFPT1,2 transfer an amino group from L-Gln to F6P to form GlcN6P
- in pathway: Synthesis of UDP-N-acetyl-glucosamine
Glucosamine-fructose 6-phosphate aminotransferases 1 and 2 (GFPT1,2) are the first and rate-limiting enzymes in the hexosamine synthesis pathway, and thus formation of hexosamines like N-acetylglucosamine (GlcNAc). These enzymes probably play a role in limiting the availability of substrates for the N- and O- linked glycosylation of proteins (McKnight et al. 1992, Oki et al. 1999). GFPT1 and 2 are required for normal functioning of neuromuscular synaptic transmission. Defects in GFPT1 lead to altered muscle fibre morphology and impaired neuromuscular junction development (Senderek et al. 2011).
Reaction - small molecule participants:
L-Glu [cytosol]
GlcN6P [cytosol]
Fru(6)P [cytosol]
L-Gln [cytosol]
Reactome.org reaction link: R-HSA-449715
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Reaction input - small molecules:
beta-D-fructofuranose 6-phosphate(2-)
L-glutamine zwitterion
Reaction output - small molecules:
L-glutamate(1-)
alpha-D-glucosamine 6-phosphate
Reactome.org link: R-HSA-449715