EC - (N-acetylneuraminyl)-galactosylglucosylceramide N-acetylgalactosaminyltransferase

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IntEnz Enzyme Nomenclature


Accepted name:
(N-acetylneuraminyl)-galactosylglucosylceramide N-acetylgalactosaminyltransferase
Other names:
GM2 synthase
UDP acetylgalactosamine-(N-acetylneuraminyl)-D-galactosyl-D-glucosylceramide acetylgalactosaminyltransferase
UDP-N-acetylgalactosamine GM3 N-acetylgalactosaminyltransferase
ganglioside GM2 synthase
ganglioside GM3 acetylgalactosaminyltransferase
uridine diphosphoacetylgalactosamine-acetylneuraminylgalactosylglucosylceramide acetylgalactosaminyltransferase
uridine diphosphoacetylgalactosamine-ganglioside GM3 acetylgalactosaminyltransferase
uridine diphosphoacetylgalactosamine-hematoside acetylgalactosaminyltransferase
asialo-GM2 synthase
UDP-N-acetyl-D-galactosamine:(N-acetylneuraminyl)-D-galactosyl-D-glucosylceramide N-acetyl-D-galactosaminyltransferase
UDP-N-acetyl-D-galactosamine:1-O-[O-(N-acetyl-α-neuraminosyl)-(2→3)-O-β-D-galactopyranosyl-(1→4)-β-D-glucopyranosyl]-ceramide 1,4-β-N-acetyl-D-galactosaminyltransferase
Systematic name:
UDP-N-acetyl-α-D-galactosamine:1-O-[O-(N-acetyl-α-neuraminosyl)-(2→3)-O-β-D-galactopyranosyl-(1→4)-β-D-glucopyranosyl]-ceramide 4-β-N-acetyl-D-galactosaminyltransferase



This enzyme catalyses the formation of the gangliosides (i.e. sialic-acid-containing glycosphingolipids) GM2, GD2 and SM2 from GM3, GD3 and SM3, respectively. Asialo-GM3 [3] and lactosylceramide [2] are also substrates, but glycoproteins and oligosaccharides are not substrates.

Links to other databases

Enzymes and pathways: NC-IUBMB , BRENDA , DIAGRAM , ExplorEnz , ENZYME@ExPASy , KEGG , MetaCyc , UniPathway
Structural data: CSA , EC2PDB
Gene Ontology: GO:0003947
CAS Registry Number: 67338-98-1


  1. Dicesare, J.L. and Dain, J.A.
    The enzymic synthesis of ganglioside. IV. UDP-N-acetylgalactosamine:(N-acetylneuraminyl)-galactosylglucosyl ceramide N-acetylgalactosaminyltransferase in rat brain.
    Biochim. Biophys. Acta 231 : 385-393 (1971). [PMID: 5554906]
  2. Pohlentz, G., Klein, D., Schwarzmann, G., Schmitz, D. and Sandhoff, K.
    Both GA2, GM2, and GD2 synthases and GM1b, GD1a, and GT1b synthases are single enzymes in Golgi vesicles from rat liver.
    Proc. Natl. Acad. Sci. USA 85 : 7044-7048 (1988). [PMID: 3140234]
  3. Hidari, J. K., Ichikawa, S., Furukawa, K., Yamasaki, M. and Hirabayashi, Y.
    β1-4N-acetylgalactosaminyltransferase can synthesize both asialoglycosphingolipid GM2 and glycosphingolipid GM2 in vitro and in vivo: isolation and characterization of a β1-4N-acetylgalactosaminyltransferase cDNA clone from rat ascites hepatoma cell line AH7974F.
    Biochem. J. 303 : 957-965 (1994). [PMID: 7980468]
  4. Hashimoto, Y., Sekine, M., Iwasaki, K. and Suzuki, A.
    Purification and characterization of UDP-N-acetylgalactosamine GM3/GD3 N-acetylgalactosaminyltransferase from mouse liver.
    J. Biol. Chem. 268 : 25857-25864 (1993). [PMID: 8245020]
  5. Nagai, K. and Ishizuka, I.
    Biosynthesis of monosulfogangliotriaosylceramide and GM2 by N-acetylgalactosaminyltransferase from rat brain.
    J. Biochem. 101 : 1115-1127 (1987). [PMID: 3115968]
  6. Furukawa, K., Takamiya, K. and Furukawa, K.
    β1,4-N-acetylgalactosaminyltransferase—GM2/GD2 synthase: a key enzyme to control the synthesis of brain-enriched complex gangliosides.
    Biochim. Biophys. Acta 1573 : 356-362 (2002). [PMID: 12417418]
  7. Yamashita, T., Wu, Y.-P., Sandhoff, R., Werth, N., Mizukami, H., Ellis, J.M., Dupree, J.L., Geyer, R., Sandhoff, K. and Proia, R.L.
    Interruption of ganglioside synthesis produces central nervous system degeneration and altered axon-glial interactions.
    Proc. Natl. Acad. Sci. USA 102 : 2725-2730 (2005). [PMID: 15710896]

[EC created 1976, modified 2006]