Reactome | B3GALNT1 transfers GalNAc to Gb3Cer to form Gb4Cer

B3GALNT1 transfers GalNAc to Gb3Cer to form Gb4Cer

Stable Identifier

R-HSA-8878914

Type

Reaction [transition]

Species

Homo sapiens

Compartment

Golgi lumen, Golgi membrane

ReviewStatus

5/5

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B3GALNT1 transfers GalNAc to Gb3Cer to form Gb4Cer

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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 Okajima, T, Nakamura, Y, Uchikawa, M, Haslam, DB, Numata, SI, Furukawa, K, Furukawa, K, Urano, T	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 Amado, M, Almeida, R, Carneiro, F, Levery, SB, Holmes, EH, Nomoto, M, Hollingsworth, MA, Hassan, H, Schwientek, T, Nielsen, PA, Bennett, EP, Clausen, H	J Biol Chem	1998