KEGG ENZYME: 2.4.1.92

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

HSA:	2583(B4GALNT1)

PTR:	452021(B4GALNT1)

PPS:	100977739(B4GALNT1)

GGO:	101142085(B4GALNT1)

PON:	100441953(B4GALNT1)

PPYG:

129009866(B4GALNT1)

NLE:	100596890(B4GALNT1)

HMH:	116480854(B4GALNT1)

SSYN:

129465668(B4GALNT1)

MCC:	715317(B4GALNT1)

MCF:	101926458(B4GALNT1)

MTHB:

126930376

MNI:	105474096(B4GALNT1)

CSAB:

103238575(B4GALNT1)

CATY:

105587249(B4GALNT1)

PANU:

101005418(B4GALNT1)

TGE:	112634643(B4GALNT1)

MLEU:

105545605(B4GALNT1)

RRO:	104682353(B4GALNT1)

RBB:	108519702(B4GALNT1)

TFN:	117089870(B4GALNT1)

PTEH:

111541797(B4GALNT1)

CANG:

105500499(B4GALNT1)

CJC:	100387150(B4GALNT1)

SBQ:	101035356(B4GALNT1)

CIMI:

108309735(B4GALNT1)

ANAN:

105729103(B4GALNT1)

CSYR:

103263166(B4GALNT1)

MMUR:

105877987(B4GALNT1)

LCAT:

123639596(B4GALNT1)

PCOQ:

105818113(B4GALNT1)

OGA:	100958711(B4GALNT1)

MMU:	14421(B4galnt1)

MCAL:

110302844(B4galnt1)

MPAH:

110326328(B4galnt1)

RNO:	64828(B4galnt1)

MCOC:

116075819(B4galnt1)

ANU:	117696826(B4galnt1)

MUN:	110561316(B4galnt1)

CGE:	100764682(B4galnt1)

MAUA:

101833950(B4galnt1)

PROB:

127215677(B4galnt1)

PLEU:

114704807(B4galnt1)

MORG:

121457348(B4galnt1)

MFOT:

126514729

AAMP:

119803561(B4galnt1)

NGI:	103742903(B4galnt1)

HGL:	101705101(B4galnt1)

CPOC:

100719351(B4galnt1)

CCAN:

109686096(B4galnt1)

DORD:

105988581(B4galnt1)

DSP:	122124387(B4galnt1)

PLOP:

125362144(B4galnt1)

NCAR:

124983376

MMMA:

107140121(B4galnt1)

OCU:

100340284

OPI:	101527960(B4GALNT1)

TUP:	102495908(B4GALNT1)

GVR:	103594212(B4GALNT1)

CFA:	481129(B4GALNT1)

CLUD:

112677966(B4GALNT1)

VVP:	112922336(B4GALNT1)

VLG:	121491928(B4GALNT1)

NPO:	129512209(B4GALNT1)

AML:	100466312(B4GALNT1)

UMR:	103656939(B4GALNT1)

UAH:	113256372(B4GALNT1)

UAR:	123787304(B4GALNT1)

ELK:

111160216

LLV:

125106163

MPUF:

101692331(B4GALNT1)

MNP:	132019847(B4GALNT1)

MLK:	131838547(B4GALNT1)

NVS:	122891153(B4GALNT1)

ORO:	101384792(B4GALNT1)

EJU:	114214987(B4GALNT1)

ZCA:	113921972(B4GALNT1)

MLX:	118008424(B4GALNT1)

NSU:	110577856(B4GALNT1)

LWW:	102747686(B4GALNT1)

FCA:	101082581(B4GALNT1)

PYU:	121040864(B4GALNT1)

PCOO:

112863779(B4GALNT1)

PBG:	122473825(B4GALNT1)

PVIV:

125170476(B4GALNT1)

LRUF:

124502179

PTG:	102953164(B4GALNT1)

PPAD:

109270387(B4GALNT1)

PUC:

125919254

AJU:

106975837

HHV:	120248004(B4GALNT1)

BTA:	520159(B4GALNT1)

BOM:	102280706(B4GALNT1)

BIU:	109559314(B4GALNT1)

BBUB:

102409613(B4GALNT1)

BBIS:

104988128(B4GALNT1)

CHX:	102173266(B4GALNT1)

OAS:	101119388(B4GALNT1)

BTAX:

128047491(B4GALNT1)

ODA:	120857502(B4GALNT1)

CCAD:

122427339(B4GALNT1)

MBEZ:

129536857(B4GALNT1)

SSC:	100516440(B4GALNT1)

CFR:	102520114(B4GALNT1)

CBAI:

105062135(B4GALNT1)

CDK:	105100336(B4GALNT1)

VPC:	102530081(B4GALNT1)

BACU:

103016988(B4GALNT1)

BMUS:

118902610(B4GALNT1)

LVE:	103088237(B4GALNT1)

OOR:	101281173(B4GALNT1)

DLE:	111173830(B4GALNT1)

PCAD:

102983599(B4GALNT1)

PSIU:

116760689(B4GALNT1)

NASI:

112394238(B4GALNT1)

ECB:	100053616(B4GALNT1)

EPZ:	103559776(B4GALNT1)

EAI:	106833013(B4GALNT1)

MYB:	102244087(B4GALNT1)

MYD:	102760120(B4GALNT1)

MMYO:

118651334(B4GALNT1)

MLF:	102434516(B4GALNT1)

MDT:	132227818(B4GALNT1)

PKL:	118730266(B4GALNT1)

EFUS:

103292474(B4GALNT1)

MNA:	107546159(B4GALNT1)

DRO:	112319125(B4GALNT1)

SHON:

118975962(B4GALNT1)

AJM:	119035675(B4GALNT1)

PDIC:

114513768(B4GALNT1)

PHAS:

123812944(B4GALNT1)

MMF:	118623520(B4GALNT1)

PPAM:

129076029(B4GALNT1)

HAI:	109390763(B4GALNT1)

RFQ:	117028118(B4GALNT1)

PALE:

102880239(B4GALNT1)

PGIG:

120604959(B4GALNT1)

PVP:	105307389(B4GALNT1)

RAY:	107501518(B4GALNT1)

MJV:	108409979(B4GALNT1)

TOD:	119248098(B4GALNT1)

SARA:

101557033(B4GALNT1)

SETR:

126022225(B4GALNT1)

LAV:	100656175(B4GALNT1)

TMU:

101357477

ETF:	101655324(B4GALNT1)

DNM:	101433475(B4GALNT1)

MDO:	100031466(B4GALNT1)

GAS:	123250467(B4GALNT1)

SHR:	100913439(B4GALNT1)

AFZ:

127538920

PCW:	110210985(B4GALNT1)

TVP:	118851807(B4GALNT1)

OAA:	114814766(B4GALNT1)

CJO:	107325778(B4GALNT1)

NMEL:

110392007

LSR:	110481834(B4GALNT1)

PMOA:

120499532(B4GALNT1)

OTC:	121334272(B4GALNT1)

PRUF:

121356353(B4GALNT1)

OMA:	130265786(B4GALNT1)

PHI:	102104849(B4GALNT1)

ZLE:	135459182(B4GALNT1)

SVG:	106862641(B4GALNT1)

SATI:

136372705(B4GALNT1)

TALA:

116959221 122153816

AGEN:

126037399

CUCA:

128849472(B4GALNT1)

RTD:	128903865(B4GALNT1)

AROW:

112961595(B4GALNT1)

ASN:	102384074(B4GALNT1)

AMJ:	102576374(B4GALNT1)

CPOO:

109324403(B4GALNT1)

CMY:	102936553(B4GALNT1)

CCAY:

125627745(B4GALNT1)

DCC:	119845978(B4GALNT1)

CPIC:

101945852(B4GALNT1)

TST:	117889048(B4GALNT1)

CABI:

116818857(B4GALNT1)

MRV:	120391165(B4GALNT1)

ACS:	100553349(b4galnt1)

ASAO:

132767715(B4GALNT1)

PVT:	110070599(B4GALNT1)

SUND:

121921249(B4GALNT1)

PBI:	103067514(B4GALNT1)

PMUR:

107294728(B4GALNT1)

CTIG:

120303310(B4GALNT1)

TSR:	106553723(B4GALNT1)

PGUT:

117666505(B4GALNT1)

APRI:

131190792(B4GALNT1)

PTEX:

113444822(B4GALNT1)

NSS:	113416181(B4GALNT1)

VKO:	123029881(B4GALNT1)

PMUA:

114592946(B4GALNT1)

PRAF:

128404881(B4GALNT1)

ZVI:	118078702(B4GALNT1)

HCG:	128342243(B4GALNT1)

GJA:	107120713(B4GALNT1)

STOW:

125428050(B4GALNT1)

EMC:	129346127(B4GALNT1)

XLA:	108709867(b4galnt1.S) 379299(b4galnt1.L)

XTR:	100145208(b4galnt1)

NPR:	108802621(B4GALNT1)

RTEM:

120929452(B4GALNT1)

BBUF:

120996074(B4GALNT1)

BGAR:

122932266(B4GALNT1)

MUO:	115465835(B4GALNT1)

GSH:	117356330(B4GALNT1)

DRE:	558684(b4galnt1a) 793635(b4galnt1b)

SRX:	107708855 107711068 107716216 107717192 107733607

SANH:

107667298 107667748 107673615 107681939 107695798

SGH:	107570598 107582708 107582953 107585207 107586541

CCAR:

109047493 109056959 109062280 109062741 109064114 109076424

CAUA:

113046700 113046702 113047614 113050355 113066542 113085425 113094168

CGIB:

127960243 128015731

PTET:

122329311(b4galnt1a) 122347215(b4galnt1b)

LROH:

127154383(b4galnt1a) 127167364(b4galnt1b)

OMC:	131531907(b4galnt1a) 131542433(b4galnt1b)

PPRM:

120463589(b4galnt1a) 120491381(b4galnt1b)

RKG:	130073611(b4galnt1a) 130087557(b4galnt1b)

MAMB:

125259537(b4galnt1a) 125273705(b4galnt1b)

CIDE:

127505109 127514749

CERY:

137009737(b4galnt1a) 137010688(b4galnt1b)

MASI:

127420175 127446676 127448647

TROS:

130556534(b4galnt1b) 130571996(b4galnt1a)

TDW:	130409795(b4galnt1a) 130425150(b4galnt1b)

MANU:

129431030(b4galnt1a) 129450290(b4galnt1b)

IPU:	108272210(b4galnt1b) 108276385 108276386 108276500

IFU:	128614781(b4galnt1b) 128619252 128619300

PHYP:

113530618 113530674 113530881 113545817(b4galnt1)

SMEO:

124390265(b4galnt1b)

TFD:	113658252(b4galnt1a) 113660538(b4galnt1b)

TVC:	132844148(b4galnt1b) 132862228(b4galnt1a)

TRN:	134316624(b4galnt1b) 134333845(b4galnt1a)

AMEX:

103039726(b4galnt1b) 103043708(b4galnt1a)

CMAO:

118808252(b4galnt1b) 118822707(b4galnt1a)

EEE:	113567787(b4galnt1) 113589183

CHAR:

105897319(b4galnt1a)

TRU:	101063785 101064428(b4galnt1)

TFS:	130524298(b4galnt1b) 130530174(b4galnt1a)

TNG:	GSTEN00027252G001 GSTEN00027254G001 GSTEN00028095G001

LCO:	104931956(b4galnt1) 104932572

NCC:	104942127 104956934

TBEN:

117490021(b4galnt1b) 117494271(b4galnt1a)

CGOB:

115008865(b4galnt1) 115010627

PGEO:

117447391(b4galnt1b) 117449311(b4galnt1a)

GACU:

117541879(b4galnt1a) 117551053(b4galnt1b)

EMAC:

134871250(b4galnt1a) 134882602(b4galnt1b)

ELY:	117257558(b4galnt1b) 117258938(b4galnt1a)

EFO:	125889274(b4galnt1b) 125891661(b4galnt1a)

PLEP:

121939030 121946469(b4galnt1a) 121950679(b4galnt1b)

SLUC:

116039006(b4galnt1a) 116041660(b4galnt1b)

ECRA:

117943914(b4galnt1b) 117947769(b4galnt1a)

ESP:	116688378(b4galnt1) 116692690

PFLV:

114554065(b4galnt1) 114559465

GAT:	120829097(b4galnt1a) 120835599(b4galnt1b)

PPUG:

119223254 119229857(b4galnt1b)

AFB:	129099881(b4galnt1a) 129105782(b4galnt1b)

CLUM:

117731019(b4galnt1b) 117734202(b4galnt1a)

PSWI:

130199399(b4galnt1a) 130208867(b4galnt1b)

MSAM:

119886148(b4galnt1b) 119905488(b4galnt1a)

SCHU:

122867054(b4galnt1b) 122883091(b4galnt1a)

CUD:	121504305 121506149(b4galnt1b) 121518213(b4galnt1a)

ALAT:

119020492(b4galnt1b) 119023434(b4galnt1a)

MZE:	101478170 101483501 112434815

ONL:	100701263(b4galnt1) 100705425

OAU:	116325820(b4galnt1a) 116333850(b4galnt1b)

OLA:	101155420(b4galnt1) 101171003

OML:	112145405(b4galnt1b) 112159647(b4galnt1a)

CSAI:

133448753(b4galnt1b) 133457191(b4galnt1a)

XMA:	102222577 102228614 102235034

XCO:	114135118 114135952 114148900

XHE:	116710988 116710989 116727168

PRET:

103465386(b4galnt1) 103467344

PFOR:

103132481 103133123 103141140 103147665

PLAI:

106939363 106941044 106950804

PMEI:

106908770 106909689 106920821 106920822

GAF:	122832697(b4galnt1a) 122834697 122834726(b4galnt1b)

PPRL:

129357775(b4galnt1b) 129372481(b4galnt1a)

CVG:	107082147(b4galnt1) 107086793

CTUL:

119776671(b4galnt1b) 119782168

GMU:	124856483(b4galnt1b) 124861330(b4galnt1a)

NFU:	107385684(b4galnt1) 107390873

KMR:	108228644(b4galnt1a) 108235553(b4galnt1b)

ALIM:

106518031(b4galnt1) 106524945

NWH:	119412559(b4galnt1b) 119416969(b4galnt1a)

AOCE:

111566651(b4galnt1) 111587243

MCEP:

124996938(b4galnt1a) 125007198(b4galnt1b)

CSEM:

103385079 103385954(b4galnt1)

POV:	109629415(b4galnt1) 109630534

SSEN:

122768416(b4galnt1b) 122777098(b4galnt1a)

HHIP:

117761296(b4galnt1b) 117765024(b4galnt1a)

HSP:	118105510(b4galnt1b) 118111613(b4galnt1a)

PPLT:

128441744(b4galnt1a) 128452214(b4galnt1b)

SMAU:

118308945(b4galnt1b) 118316827(b4galnt1a)

LCF:	108876488(b4galnt1a) 108881464(b4galnt1b)

SDU:	111219289 111221963(b4galnt1)

SLAL:

111667834(b4galnt1) 111668982

XGL:	120783424(b4galnt1b) 120804023(b4galnt1a)

HCQ:	109522137(b4galnt1) 109531384

SSCV:

125976677 125988023

SBIA:

133505580(b4galnt1a) 133507139(b4galnt1b)

PEE:	133402341(b4galnt1a) 133409129(b4galnt1b)

PTAO:

133478538(b4galnt1a) 133483763(b4galnt1b)

BPEC:

110164780 110168375(b4galnt1)

MALB:

109968366 109972083

BSPL:

114856333(b4galnt1b) 114859002(b4galnt1a)

SJO:	128356009(b4galnt1b) 128357945(b4galnt1a)

SASA:

100306792(b4gn1) 106565528 106572659

STRU:

115150577 115165495 115207831

OTW:	112216357 112217564(b4galnt1b) 112254603

OMY:	110494320 110528114(b4galnt1b) 110532694

OGO:	124003500 124019822(b4galnt1b) 124032285

ONE:	115125666 115139823 115143172

OKI:	109891138 109904750 109907071

OKE:	118376214 118388567 118400293(b4galnt1b)

SALP:

111966771 111966811 111970082(b4galnt1)

SNH:	120058804 120061253 120065186

CCLU:

121537887 121575259 121577619

ELS:	105005742 105013542 105017218(b4galnt1)

SFM:	108918587(b4galnt1) 108939037

PKI:	111851086 111854415(b4galnt1) 111860628

AANG:

118207800 118211247(b4galnt1b)

LOC:	102693606(b4galnt1)

PSPA:

121308998(b4galnt1b)

ARUT:

117401004 117967072

PSEX:

120524830(b4galnt1b)

LCM:	102353417 102354362(B4GALNT1)

CMK:	103189519 103189521

CPLA:

122543394 122543431

HOC:	132805703 132805729

LERI:

129714719 129714721(b4galnt1b)

PMRN:

116944255 116946880 116946891 116951224

LRJ:	133344966 133344967 133345076

BFO:	118403673 118404274 118409104 118410769 118413253 118413880 118413881 118416384 118420990 118423730 118424548 118427545 118429972 118431016 118431239

BBEL:

109462277 109467557 109468930 109469051 109469639 109470355 109471463 109472103 109472932 109473331 109474378 109474463 109475330 109478673 109482281 109484300 109486291

SKO:	100366571 100366802 100367188 100367258 100367410 100367566 100368131 100369117 100369900 100372465 100374186 100377850 100377910 100378876 102804391

LAK:

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

DBGET integrated database retrieval system