KEGG   ENZYME: 2.4.1.92Help
Entry
EC 2.4.1.92                 Enzyme                                 

Name (N-acetylneuraminyl)-galactosylglucosylceramide
N-acetylgalactosaminyltransferase;
uridine diphosphoacetylgalactosamine-ganglioside GM3
acetylgalactosaminyltransferase;
ganglioside GM2 synthase;
ganglioside GM3 acetylgalactosaminyltransferase;
GM2 synthase;
UDP
acetylgalactosamine-(N-acetylneuraminyl)-D-galactosyl-D-
glucosylceramide acetylgalactosaminyltransferase;
UDP-N-acetyl-D-galactosamine:1-O-[O-(N-acetyl-alpha-neuraminosyl)-
(2->3)-O-beta-D-galactopyranosyl-(1->4)-beta-D-glucopyranosyl]-
ceramide 1,4-beta-N-acetyl-D-galactosaminyltransferase
acetylgalactosaminyltransferase;
UDP-N-acetylgalactosamine GM3 N-acetylgalactosaminyltransferase;
uridine
diphosphoacetylgalactosamine-
acetylneuraminylgalactosylglucosylceramide
acetylgalactosaminyltransferase;
uridine diphosphoacetylgalactosamine-hematoside
acetylgalactosaminyltransferase;
GM2/GD2-synthase;
beta-1,4N-aetylgalactosaminyltransferase;
asialo-GM2 synthase;
GalNAc-T;
UDP-N-acetyl-D-galactosamine:(N-acetylneuraminyl)-D-galactosyl-D-
glucosylceramide N-acetyl-D-galactosaminyltransferase
Class Transferases;
Glycosyltransferases;
Hexosyltransferases
BRITE hierarchy
Sysname UDP-N-acetyl-D-galactosamine:1-O-[O-(N-acetyl-alpha-neuraminosyl)-(2
->3)-O-beta-D-galactopyranosyl-(1->4)-beta-D-glucopyranosyl]-ceramid
e 4-beta-N-acetyl-D-galactosaminyltransferase
Reaction(IUBMB) UDP-N-acetyl-D-galactosamine +
1-O-[O-(N-acetyl-alpha-neuraminosyl)-(2->3)-O-beta-D-
galactopyranosyl-(1->4)-beta-D-glucopyranosyl]-ceramide = UDP +
1-O-[O-2-(acetylamino)-2-deoxy-beta-D-galactopyranosyl-(1->4)-O-[N-
acetyl-alpha-neuraminosyl-(2->3)]-O-beta-D-galactopyranosyl-(1->4)-
beta-D-glucopyranosyl]-ceramide [RN:R04584 R05939]
Reaction(KEGG) R04584 R05939(G);
(other) R03490 R05109 R05938(G) R05946(G) R05952(G)
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Substrate UDP-N-acetyl-D-galactosamine [CPD:C00203];
1-O-[O-(N-acetyl-alpha-neuraminosyl)-(2->3)-O-beta-D-
galactopyranosyl-(1->4)-beta-D-glucopyranosyl]-ceramide
Product UDP [CPD:C00015];
1-O-[O-2-(acetylamino)-2-deoxy-beta-D-galactopyranosyl-(1->4)-O-[N-
acetyl-alpha-neuraminosyl-(2->3)]-O-beta-D-galactopyranosyl-(1->4)-
beta-D-glucopyranosyl]-ceramide
Comment 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.
Pathway PATH: ec00604  Glycosphingolipid biosynthesis - ganglio series
PATH: ec01100  Metabolic pathways
Orthology KO: K00725  (N-Acetylneuraminyl)-galactosylglucosylceramide
            N-acetylgalactosaminyltransferase
Genes HSA: 2583(B4GALNT1)
PTR: 452021(B4GALNT1)
MCC: 715317(B4GALNT1)
MMU: 14421(B4galnt1)
RNO: 64828(B4galnt1)
CFA: 481129(B4GALNT1)
BTA: 520159(B4GALNT1)
ECB: 100053616
MDO: 100031466
XLA: 379299(MGC53523)
XTR: 100145208
DRE: 793635
Taxonomy
Reference
  Authors
  Title


  Journal
  Organism
1  [PMID:5554906]
Dicesare JL, Dain JA.
The enzymic synthesis of ganglioside. IV. UDP-N-acetylgalactosamine:
(N-acetylneuraminyl)-galactosylglucosyl ceramide
N-acetylgalactosaminyltransferase in rat brain.
Biochim. Biophys. Acta. 231 (1971) 385-93.
Rattus norvegicus [GN:rno]
Reference
  Authors
  Title

  Journal
  Organism
2  [PMID:3140234]
Pohlentz G, Klein D, Schwarzmann G, Schmitz D, 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. U. S. A. 85 (1988) 7044-8.
Rattus norvegicus [GN:rno]
Reference
  Authors
  Title




  Journal
  Organism
3  [PMID:7980468]
Hidari JK, Ichikawa S, Furukawa K, Yamasaki M, Hirabayashi Y.
beta 1-4N-acetylgalactosaminyltransferase can synthesize both
asialoglycosphingolipid GM2 and glycosphingolipid GM2 in vitro and
in vivo: isolation and characterization of a beta
1-4N-acetylgalactosaminyltransferase cDNA clone from rat ascites
hepatoma cell line AH7974F.
Biochem. J. 303 ( Pt 3) (1994) 957-65.
Rattus norvegicus [GN:rno]
Reference
  Authors
  Title

  Journal
  Organism
4  [PMID:8245020]
Hashimoto Y, Sekine M, Iwasaki K, Suzuki A.
Purification and characterization of UDP-N-acetylgalactosamine
GM3/GD3 N-acetylgalactosaminyltransferase from mouse liver.
J. Biol. Chem. 268 (1993) 25857-64.
Mus musculus [GN:mmu]
Reference
  Authors
  Title

  Journal
  Organism
5  [PMID:3115968]
Nagai K, Ishizuka I.
Biosynthesis of monosulfogangliotriaosylceramide and GM2 by
N-acetylgalactosaminyltransferase from rat brain.
J. Biochem. (Tokyo). 101 (1987) 1115-27.
Rattus norvegicus [GN:rno]
Reference
  Authors
  Title


  Journal
  Organism
6  [PMID:12417418]
Furukawa K, Takamiya K, Furukawa K.
Beta1,4-N-acetylgalactosaminyltransferase--GM2/GD2 synthase: a key
enzyme to control the synthesis of brain-enriched complex
gangliosides.
Biochim. Biophys. Acta. 1573 (2002) 356-62.
Mus musculus [GN:mmu]
Reference
  Authors

  Title

  Journal
  Organism
7  [PMID:15710896]
Yamashita T, Wu YP, Sandhoff R, Werth N, Mizukami H, Ellis JM,
Dupree JL, Geyer R, Sandhoff K, Proia RL.
Interruption of ganglioside synthesis produces central nervous
system degeneration and altered axon-glial interactions.
Proc. Natl. Acad. Sci. U. S. A. 102 (2005) 2725-30.
Mus musculus [GN:mmu]
Other DBs ExplorEnz - The Enzyme Database: 2.4.1.92
IUBMB Enzyme Nomenclature: 2.4.1.92
ExPASy - ENZYME nomenclature database: 2.4.1.92
BRENDA, the Enzyme Database: 2.4.1.92
CAS: 67338-98-1

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