KEGG   ENZYME: 2.4.1.92
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-acetylgalactosaminyltransferase;
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
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) R12962
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
ec00600  Sphingolipid metabolism
ec00604  Glycosphingolipid biosynthesis - ganglio series
ec01100  Metabolic pathways
Orthology
K00725  (N-Acetylneuraminyl)-galactosylglucosylceramide N-acetylgalactosaminyltransferase
Genes
HSA2583(B4GALNT1)
PTR452021(B4GALNT1)
PPS100977739(B4GALNT1)
GGO101142085(B4GALNT1)
PON100441953(B4GALNT1)
NLE100596890(B4GALNT1)
MCC715317(B4GALNT1)
MCF101926458(B4GALNT1)
CSAB103238575(B4GALNT1)
CATY105587249(B4GALNT1)
PANU101005418(B4GALNT1)
TGE112634643(B4GALNT1)
RRO104682353(B4GALNT1)
RBB108519702(B4GALNT1)
TFN117089870(B4GALNT1)
PTEH111541797(B4GALNT1)
CJC100387150(B4GALNT1)
SBQ101035356(B4GALNT1)
CSYR103263166(B4GALNT1)
MMUR105877987(B4GALNT1)
LCAT123639596(B4GALNT1)
OGA100958711(B4GALNT1)
MMU14421(B4galnt1)
MCAL110302844(B4galnt1)
MPAH110326328(B4galnt1)
RNO64828(B4galnt1)
MCOC116075819(B4galnt1)
MUN110561316(B4galnt1)
CGE100764682(B4galnt1)
MAUA101833950(B4galnt1)
PLEU114704807(B4galnt1)
MORG121457348(B4galnt1)
AAMP119803561(B4galnt1)
NGI103742903(B4galnt1)
HGL101705101(B4galnt1)
CPOC100719351(B4galnt1)
CCAN109686096(B4galnt1)
DORD105988581(B4galnt1)
DSP122124387(B4galnt1)
NCAR124983376
OCU100340284(B4GALNT1)
OPI101527960(B4GALNT1)
TUP102495908(B4GALNT1)
CFA481129(B4GALNT1)
CLUD112677966(B4GALNT1)
VVP112922336(B4GALNT1)
VLG121491928(B4GALNT1)
AML100466312(B4GALNT1)
UMR103656939(B4GALNT1)
UAH113256372(B4GALNT1)
UAR123787304(B4GALNT1)
ELK111160216
LLV125106163
MPUF101692331(B4GALNT1)
ORO101384792(B4GALNT1)
EJU114214987(B4GALNT1)
ZCA113921972(B4GALNT1)
MLX118008424(B4GALNT1)
FCA101082581(B4GALNT1)
PYU121040864(B4GALNT1)
PBG122473825(B4GALNT1)
PTG102953164(B4GALNT1)
PPAD109270387(B4GALNT1)
AJU106975837(B4GALNT1)
HHV120248004(B4GALNT1)
BTA520159(B4GALNT1)
BOM102280706(B4GALNT1)
BIU109559314(B4GALNT1)
BBUB102409613(B4GALNT1)
CHX102173266(B4GALNT1)
OAS101119388(B4GALNT1)
ODA120857502(B4GALNT1)
CCAD122427339(B4GALNT1)
SSC100516440(B4GALNT1)
CFR102520114(B4GALNT1)
CBAI105062135(B4GALNT1)
CDK105100336(B4GALNT1)
VPC102530081(B4GALNT1)
BACU103016988(B4GALNT1)
LVE103088237(B4GALNT1)
OOR101281173(B4GALNT1)
DLE111173830(B4GALNT1)
PCAD102983599(B4GALNT1)
PSIU116760689(B4GALNT1)
ECB100053616(B4GALNT1)
EPZ103559776(B4GALNT1)
EAI106833013(B4GALNT1)
MYB102244087(B4GALNT1)
MYD102760120(B4GALNT1)
MMYO118651334(B4GALNT1)
MLF102434516(B4GALNT1)
MNA107546159(B4GALNT1)
PKL118730266(B4GALNT1)
HAI109390763(B4GALNT1)
DRO112319125(B4GALNT1)
SHON118975962(B4GALNT1)
AJM119035675(B4GALNT1)
PDIC114513768(B4GALNT1)
PHAS123812944(B4GALNT1)
MMF118623520(B4GALNT1)
RFQ117028118(B4GALNT1)
PALE102880239(B4GALNT1)
PGIG120604959(B4GALNT1)
PVP105307389(B4GALNT1)
RAY107501518(B4GALNT1)
MJV108409979(B4GALNT1)
TOD119248098(B4GALNT1)
SARA101557033(B4GALNT1)
LAV100656175(B4GALNT1)
TMU101357477
DNM101433475(B4GALNT1)
MDO100031466(B4GALNT1)
GAS123250467(B4GALNT1)
SHR100913439(B4GALNT1)
PCW110210985(B4GALNT1)
OAA114814766(B4GALNT1)
CJO107325778(B4GALNT1)
NMEL110392007
LSR110481834(B4GALNT1)
PMOA120499532(B4GALNT1)
OTC121334272(B4GALNT1)
PRUF121356353(B4GALNT1)
PHI102104849(B4GALNT1)
TALA116959221 122153816
AROW112961595(B4GALNT1)
ASN102384074(B4GALNT1)
AMJ102576374(B4GALNT1)
CPOO109324403(B4GALNT1)
CMY102936553(B4GALNT1)
CPIC101945852(B4GALNT1)
TST117889048(B4GALNT1)
CABI116818857(B4GALNT1)
MRV120391165(B4GALNT1)
ACS100553349(b4galnt1)
PVT110070599(B4GALNT1)
SUND121921249(B4GALNT1)
PBI103067514(B4GALNT1)
PMUR107294728(B4GALNT1)
TSR106553723(B4GALNT1)
PGUT117666505(B4GALNT1)
VKO123029881(B4GALNT1)
PMUA114592946(B4GALNT1)
ZVI118078702(B4GALNT1)
GJA107120713(B4GALNT1)
STOW125428050(B4GALNT1)
XLA108709867(b4galnt1.S) 379299(b4galnt1.L)
XTR100145208(b4galnt1)
NPR108802621(B4GALNT1)
RTEM120929452(B4GALNT1)
BBUF120996074(B4GALNT1)
BGAR122932266(B4GALNT1)
DRE558684(b4galnt1a) 793635(b4galnt1b)
SRX107708855 107711068 107716216 107717192 107733607
SANH107667298 107667748 107673615 107681939 107695798
SGH107570598 107582708 107582953 107585207 107586541
CCAR109047493 109056959 109062280 109062741 109064114 109076424
CAUA113046700 113046702 113047614 113050355 113066542 113085425 113094168
PPRM120463589(b4galnt1a) 120491381(b4galnt1b)
MAMB125259537(b4galnt1a) 125273705(b4galnt1b)
IPU108272210(b4galnt1b) 108276385 108276386 108276500
PHYP113530618 113530674 113530881 113545817(b4galnt1)
SMEO124390265(b4galnt1b)
TFD113658252(b4galnt1a) 113660538(b4galnt1b)
AMEX103039726(b4galnt1) 103043708
EEE113567787(b4galnt1) 113589183
TRU101063785 101064428(b4galnt1)
TNGGSTEN00027252G001 GSTEN00027254G001 GSTEN00028095G001
LCO104931956(b4galnt1) 104932572
NCC104942127 104956934
CGOB115008865(b4galnt1) 115010627
ELY117257558(b4galnt1b) 117258938(b4galnt1a)
EFO125889274(b4galnt1b) 125891661(b4galnt1a)
PLEP121939030 121946469(b4galnt1a) 121950679(b4galnt1b)
SLUC116039006(b4galnt1a) 116041660(b4galnt1b)
ECRA117943914(b4galnt1b) 117947769(b4galnt1a)
PFLV114554065(b4galnt1) 114559465
GAT120829097(b4galnt1a) 120835599(b4galnt1b)
PPUG119223254 119229857(b4galnt1b)
MSAM119886148(b4galnt1b) 119905488(b4galnt1a)
CUD121504305 121506149(b4galnt1b) 121518213(b4galnt1a)
ALAT119020492(b4galnt1b) 119023434(b4galnt1a)
MZE101478170 101483501 112434815
ONL100701263(b4galnt1) 100705425
OAU116325820(b4galnt1a) 116333850(b4galnt1b)
OLA101155420(b4galnt1) 101171003
OML112145405(b4galnt1b) 112159647(b4galnt1a)
XMA102222577 102228614 102235034
XCO114135118 114135952 114148900
XHE116710988 116710989 116727168
PRET103465386(b4galnt1) 103467344
PFOR103132481 103133123 103141140 103147665
PLAI106939363 106941044 106950804
PMEI106908770 106909689 106920821 106920822
GAF122832697(b4galnt1a) 122834697 122834726(b4galnt1b)
CVG107082147(b4galnt1) 107086793
CTUL119776671(b4galnt1b) 119782168
GMU124856483(b4galnt1b) 124861330(b4galnt1a)
NFU107385684(b4galnt1) 107390873
KMR108228644(b4galnt1a) 108235553(b4galnt1b)
ALIM106518031(b4galnt1) 106524945
NWH119412559(b4galnt1b) 119416969(b4galnt1a)
AOCE111566651(b4galnt1) 111587243
MCEP124996938(b4galnt1a) 125007198(b4galnt1b)
CSEM103385079 103385954(b4galnt1)
POV109629415(b4galnt1) 109630534
SSEN122768416(b4galnt1b) 122777098(b4galnt1a)
HHIP117761296(b4galnt1b) 117765024(b4galnt1a)
HSP118105510(b4galnt1b) 118111613(b4galnt1a)
LCF108876488 108881464(b4galnt1)
SDU111219289 111221963(b4galnt1)
SLAL111667834(b4galnt1) 111668982
XGL120783424(b4galnt1b) 120804023(b4galnt1a)
HCQ109522137(b4galnt1) 109531384
BPEC110164780 110168375(b4galnt1)
MALB109968366 109972083
BSPL114856333(b4galnt1b) 114859002(b4galnt1a)
SASA100306792(b4gn1) 106565528 106572659
OTW112216357 112217564(b4galnt1b) 112254603
OMY110494320 110528114(b4galnt1b) 110532694
OGO124003500 124019822(b4galnt1b) 124032285
ONE115125666 115139823 115143172
SALP111966771 111966811 111970082(b4galnt1)
SNH120058804 120061253 120065186
CCLU121537887 121575259 121577619
ELS105005742 105013542 105017218(b4galnt1)
SFM108918587(b4galnt1) 108939037
PKI111851086 111854415(b4galnt1) 111860628
AANG118207800 118211247(b4galnt1b)
LOC102693606(b4galnt1)
PSPA121308998(b4galnt1b)
ARUT117401004 117967072
LCM102353417 102354362(B4GALNT1)
CMK103189519 103189521
RTP109925717(b4galnt1)
BFO118403673 118404274 118409104 118410769 118413253 118413880 118413881 118416384 118420990 118423730 118424548 118427545 118429972 118431016 118431239
BBEL109462277 109467557 109468930 109469051 109469639 109470355 109471463 109472103 109472932 109473331 109474378 109474463 109475330 109478673 109482281 109484300 109486291
SKO100366571 100366802 100367188 100367258 100367410 100367566 100368131 100369117 100369599 100369900 100372465 100374186 100377850 100377910 100378728 100378876 102804391
LAK106152907 106162682
 » show all
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 231:385-93 (1971)
DOI:10.1016/0005-2760(71)90151-2
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 85:7044-8 (1988)
DOI:10.1073/pnas.85.19.7044
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):957-65 (1994)
DOI:10.1042/bj3030957
  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 268:25857-64 (1993)
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) 101:1115-27 (1987)
DOI:10.1093/oxfordjournals.jbchem.a121976
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 1573:356-62 (2002)
DOI:10.1016/S0304-4165(02)00403-8
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 102:2725-30 (2005)
DOI:10.1073/pnas.0407785102
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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