B3GALNT1 transfers GalNAc to Gb3Cer to form Gb4Cer

Stable Identifier
Reaction [transition]
Homo sapiens
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Globosides are a type of glycosphingolipid with more than one sugar as the side chain of ceramide, usually a combination of N-acetylgalactosamine, D-glucose or D-galactose. They are components of cellular membranes, especially of the kidneys, red blood cells, blood serum, liver and spleen. Globoside synthesis (and therefore accummulation) increases in pathological conditions including neurodegenerative disorders, immune diseases and cancer. UDP-GalNAc:beta-1,3-N-acetylgalactosaminyltransferase 1 (B3GALNT1, B3GALT3) is a Golgi membrane-associated protein that can add N-acetylgalactosamine (GalNAc) to globotriaosylceramide (Gb3Cer) to form Gb4Cer (Okajima et al. 2000, Amado et al. 1998).

Literature References
PubMed ID Title Journal Year
10993897 Expression cloning of human globoside synthase cDNAs. Identification of beta 3Gal-T3 as UDP-N-acetylgalactosamine:globotriaosylceramide beta 1,3-N-acetylgalactosaminyltransferase

Nakamura, Y, Numata, SI, Haslam, DB, Urano, T, Uchikawa, M, Okajima, T, Furukawa, K, Furukawa, K

J. Biol. Chem. 2000
9582303 A family of human beta3-galactosyltransferases. Characterization of four members of a UDP-galactose:beta-N-acetyl-glucosamine/beta-nacetyl-galactosamine beta-1,3-galactosyltransferase family

Holmes, EH, Almeida, R, Bennett, EP, Nomoto, M, Amado, M, Hassan, H, Carneiro, F, Schwientek, T, Hollingsworth, MA, Nielsen, PA, Levery, SB, Clausen, H

J Biol Chem 1998
Catalyst Activity

galactosylgalactosylglucosylceramide beta-D-acetylgalactosaminyltransferase activity of B3GALNT1 [Golgi membrane]

Orthologous Events
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