Reactome | GFPT1,2 transfer an amino group from L-Gln to F6P to form GlcN6P

GFPT1,2 transfer an amino group from L-Gln to F6P to form GlcN6P

Stable Identifier

R-HSA-449715

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol

Synonyms

Formation of glucosamine 6-phosphate from fructose 6-phosphate and glutamine

ReviewStatus

5/5

Locations in the PathwayBrowser

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Metabolism of proteins (Homo sapiens)

- Post-translational protein modification (Homo sapiens)
  - Asparagine N-linked glycosylation (Homo sapiens)
    - Biosynthesis of the N-glycan precursor (dolichol lipid-linked oligosaccharide, LLO) and transfer to a nascent protein (Homo sapiens)
      - Synthesis of substrates in N-glycan biosythesis (Homo sapiens)
        
        Synthesis of UDP-N-acetyl-glucosamine (Homo sapiens)
        
        GFPT1,2 transfer an amino group from L-Gln to F6P to form GlcN6P (Homo sapiens)

General

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GFPT1,2 transfer an amino group from L-Gln to F6P to form GlcN6P

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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).

Literature References

PubMed ID	Title	Journal	Year
21310273	Hexosamine biosynthetic pathway mutations cause neuromuscular transmission defect Abicht, A, Dusl, M, Nilipour, Y, Straub, V, Colomer, J, Laval, SH, Voit, T, Kariminejad, A, Müller, JS, Steinlein, OK, Strom, TM, Maxwell, S, Mallebrera, CJ, Palace, J, Guergueltcheva, V, Schoser, B, Krause, S, Bushby, K, Sieb, JP, Lochmüller, H, Beeson, D, Oldfors, A, Senderek, J, von der Hagen, M, Bozorgmehr, B, Muelas, N, Herrmann, R, Schlotter, B, Nascimento, A, Kirschner, J, Lindbergh, C, Vilchez, JJ, Najmabadi, H, Hübner, A, Diepolder, I, Urtizberea, A, Nafissi, S, Cossins, J, Rodolico, C	Am J Hum Genet	2011
1460020	Molecular cloning, cDNA sequence, and bacterial expression of human glutamine:fructose-6-phosphate amidotransferase O'Hara, PJ, Traxinger, RR, Marshall, S, McKnight, GL, Sheppard, PO, Mathewes, SL, Mudri, SL	J Biol Chem	1992
10198162	cDNA cloning and mapping of a novel subtype of glutamine:fructose-6-phosphate amidotransferase (GFAT2) in human and mouse Okada, M, Kuromitsu, J, Yamazaki, K, Oki, T, Tanaka, I	Genomics	1999