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-neuraminyl)-(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;
UDP-N-acetyl-D-galactosamine:1-O-[O-(N-acetyl-alpha-neuraminyl)-(2->3)-O-beta-D-galactopyranosyl-(1->4)-beta-D-glucopyranosyl]-ceramide 4-beta-N-acetyl-D-galactosaminyltransferase
Class
Transferases;
Glycosyltransferases;
Hexosyltransferases
BRITE hierarchy
Sysname
UDP-N-acetyl-alpha-D-galactosamine:O-(N-acetyl-alpha-neuraminyl)-(2->3)-O-beta-D-galactopyranosyl-(1->4)-beta-D-glucopyranosyl-(1<->1)-ceramide 4-beta-N-acetyl-D-galactosaminyltransferase
Reaction(IUBMB)
UDP-N-acetyl-alpha-D-galactosamine + O-(N-acetyl-alpha-neuraminyl)-(2->3)-O-beta-D-galactopyranosyl-(1->4)-beta-D-glucopyranosyl-(1<->1)-ceramide = UDP + O-2-(acetylamino)-2-deoxy-beta-D-galactopyranosyl-(1->4)-O-[N-acetyl-alpha-neuraminyl-(2->3)]-O-beta-D-galactopyranosyl-(1->4)-beta-D-glucopyranosyl-(1<->1)-ceramide [RN:R04584 R05939]
Reaction(KEGG)
R04584 R05939(G);
(other) R03490 R05109 R05938(G) R05946(G) R05952(G)
Show
Substrate
UDP-N-acetyl-alpha-D-galactosamine [CPD:C00203];
O-(N-acetyl-alpha-neuraminyl)-(2->3)-O-beta-D-galactopyranosyl-(1->4)-beta-D-glucopyranosyl-(1<->1)-ceramide
Product
UDP [CPD:C00015];
O-2-(acetylamino)-2-deoxy-beta-D-galactopyranosyl-(1->4)-O-[N-acetyl-alpha-neuraminyl-(2->3)]-O-beta-D-galactopyranosyl-(1->4)-beta-D-glucopyranosyl-(1<->1)-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.
History
EC 2.4.1.92 created 1976, modified 2006
Pathway
Glycosphingolipid biosynthesis - ganglio series
Metabolic pathways
Orthology
K00725  
(N-Acetylneuraminyl)-galactosylglucosylceramide N-acetylgalactosaminyltransferase
Genes
HSA: 
2583(B4GALNT1)
PTR: 
452021(B4GALNT1)
PPS: 
100977739(B4GALNT1)
GGO: 
101142085(B4GALNT1)
PON: 
100441953(B4GALNT1)
MCC: 
715317(B4GALNT1)
MCF: 
MMU: 
14421(B4galnt1)
RNO: 
64828(B4galnt1)
CGE: 
100764682(B4galnt1)
HGL: 
101705101(B4galnt1)
TUP: 
102495908(B4GALNT1)
CFA: 
481129(B4GALNT1)
AML: 
FCA: 
101082581(B4GALNT1)
PTG: 
102953164(B4GALNT1)
BTA: 
520159(B4GALNT1)
BOM: 
102280706(B4GALNT1)
PHD: 
102317041(B4GALNT1)
CHX: 
102173266(B4GALNT1)
SSC: 
100516440(B4GALNT1)
CFR: 
102520114(B4GALNT1)
BACU: 
103016988(B4GALNT1)
LVE: 
103088237(B4GALNT1)
ECB: 
100053616(B4GALNT1)
MYB: 
102244087(B4GALNT1)
MYD: 
102760120(B4GALNT1)
PALE: 
102880239(B4GALNT1)
MDO: 
100031466(B4GALNT1)
SHR: 
100913439(B4GALNT1)
PHI: 
102104849(B4GALNT1)
ASN: 
102384074(B4GALNT1)
AMJ: 
102576374(B4GALNT1)
CMY: 
102936553(B4GALNT1)
ACS: 
100553349(b4galnt1)
PBI: 
103067514(B4GALNT1)
XLA: 
379299(b4galnt1)
XTR: 
100145208(b4galnt1)
DRE: 
TRU: 
MZE: 
OLA: 
XMA: 
LCM: 
CMK: 
BFO: 
 » show all
Taxonomy
Reference
1  [PMID:5554906]
  Authors
Dicesare JL, Dain JA.
  Title
The enzymic synthesis of ganglioside. IV. UDP-N-acetylgalactosamine: (N-acetylneuraminyl)-galactosylglucosyl ceramide N-acetylgalactosaminyltransferase in rat brain.
  Journal
Biochim. Biophys. Acta. 231 (1971) 385-93.
  Organism
Rattus norvegicus
Reference
2  [PMID:3140234]
  Authors
Pohlentz G, Klein D, Schwarzmann G, Schmitz D, Sandhoff K.
  Title
Both GA2, GM2, and GD2 synthases and GM1b, GD1a, and GT1b synthases are single enzymes in Golgi vesicles from rat liver.
  Journal
Proc. Natl. Acad. Sci. U. S. A. 85 (1988) 7044-8.
  Organism
Rattus norvegicus
Reference
3  [PMID:7980468]
  Authors
Hidari JK, Ichikawa S, Furukawa K, Yamasaki M, Hirabayashi Y.
  Title
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.
  Journal
Biochem. J. 303 ( Pt 3) (1994) 957-65.
  Organism
Rattus norvegicus
  Sequence
[rno:64828]
Reference
4  [PMID:8245020]
  Authors
Hashimoto Y, Sekine M, Iwasaki K, Suzuki A.
  Title
Purification and characterization of UDP-N-acetylgalactosamine GM3/GD3 N-acetylgalactosaminyltransferase from mouse liver.
  Journal
J. Biol. Chem. 268 (1993) 25857-64.
  Organism
Mus musculus
Reference
5  [PMID:3115968]
  Authors
Nagai K, Ishizuka I.
  Title
Biosynthesis of monosulfogangliotriaosylceramide and GM2 by N-acetylgalactosaminyltransferase from rat brain.
  Journal
J. Biochem. (Tokyo). 101 (1987) 1115-27.
  Organism
Rattus norvegicus
Reference
6  [PMID:12417418]
  Authors
Furukawa K, Takamiya K, Furukawa K.
  Title
Beta1,4-N-acetylgalactosaminyltransferase--GM2/GD2 synthase: a key enzyme to control the synthesis of brain-enriched complex gangliosides.
  Journal
Biochim. Biophys. Acta. 1573 (2002) 356-62.
  Organism
Mus musculus
Reference
7  [PMID:15710896]
  Authors
Yamashita T, Wu YP, Sandhoff R, Werth N, Mizukami H, Ellis JM, Dupree JL, Geyer R, Sandhoff K, Proia RL.
  Title
Interruption of ganglioside synthesis produces central nervous system degeneration and altered axon-glial interactions.
  Journal
Proc. Natl. Acad. Sci. U. S. A. 102 (2005) 2725-30.
  Organism
Mus musculus
Other DBs
ExplorEnz - The Enzyme Database: 
IUBMB Enzyme Nomenclature: 
ExPASy - ENZYME nomenclature database: 
BRENDA, the Enzyme Database: 
CAS: 
67338-98-1

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