KEGG   ENZYME: 2.4.1.135
Entry
EC 2.4.1.135                Enzyme                                 

Name
galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase;
glucuronosyltransferase I;
uridine diphosphate glucuronic acid:acceptor glucuronosyltransferase;
UDP-glucuronate:3-beta-D-galactosyl-4-beta-D-galactosyl-O-beta-D-xylosyl-protein D-glucuronosyltransferase;
UDP-glucuronate:3-beta-D-galactosyl-4-beta-D-galactosyl-O-beta-D-xylosylprotein D-glucuronosyltransferase
Class
Transferases;
Glycosyltransferases;
Hexosyltransferases
Sysname
UDP-alpha-D-glucuronate:[protein]-3-O-(beta-D-galactosyl-(1->3)-beta-D-galactosyl-(1->4)-beta-D-xylosyl)-L-serine D-glucuronosyltransferase (configuration-inverting)
Reaction(IUBMB)
UDP-alpha-D-glucuronate + [protein]-3-O-(beta-D-galactosyl-(1->3)-beta-D-galactosyl-(1->4)-beta-D-xylosyl)-L-serine = UDP + [protein]-3-O-(beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl)-L-serine [RN:R04607 R05928]
Reaction(KEGG)
R04607 R05928(G);
(other) R12482(G)
Substrate
UDP-alpha-D-glucuronate [CPD:C00167];
[protein]-3-O-(beta-D-galactosyl-(1->3)-beta-D-galactosyl-(1->4)-beta-D-xylosyl)-L-serine [CPD:C04825]
Product
UDP [CPD:C00015];
[protein]-3-O-(beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl)-L-serine [CPD:C04903]
Comment
Involved in the biosynthesis of the linkage region of glycosaminoglycan chains as part of proteoglycan biosynthesis (chondroitin, dermatan and heparan sulfates). Requires Mn2+.
History
EC 2.4.1.135 created 1984, modified 2002, modified 2016
Pathway
ec00515  Mannose type O-glycan biosynthesis
ec00532  Glycosaminoglycan biosynthesis - chondroitin sulfate / dermatan sulfate
ec00534  Glycosaminoglycan biosynthesis - heparan sulfate / heparin
ec01100  Metabolic pathways
Orthology
K00735  galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase 1
K10157  galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase 2
K10158  galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase 3
K10812  beta-1,3-glucuronyltransferase S
K23840  beta-1,3-glucuronyltransferase P
Genes
HSA: 135152(B3GAT2) 26229(B3GAT3) 27087(B3GAT1)
PTR: 462813(B3GAT2) 466859(B3GAT1) 740388(B3GAT3)
PPS: 100980067(B3GAT1) 100993761(B3GAT2) 103783318(B3GAT3)
GGO: 101132051(B3GAT2) 101142201(B3GAT1) 109028750(B3GAT3)
PON: 100448790(B3GAT1) 100449395(B3GAT2) 100458701(B3GAT3)
NLE: 100584249(B3GAT1) 100592146(B3GAT3) 100603558(B3GAT2)
MCC: 695293(B3GAT1) 717157(B3GAT2) 718744(B3GAT3)
MCF: 101867087(B3GAT3) 102118326(B3GAT2) 102142387(B3GAT1)
CSAB: 103234006(B3GAT3) 103243928(B3GAT2) 103248899(B3GAT1)
CATY: 105573543(B3GAT1) 105577553(B3GAT3) 105589182(B3GAT2)
PANU: 101006512(B3GAT2) 101012905(B3GAT3) 101022978(B3GAT1)
RRO: 104661430(B3GAT1) 104668762(B3GAT2) 104679349(B3GAT3)
RBB: 108529217(B3GAT2) 108529702(B3GAT1) 108533582(B3GAT3)
TFN: 117081104(B3GAT3) 117081774(B3GAT1) 117087431(B3GAT2)
PTEH: 111522093(B3GAT1) 111524301 111528416(B3GAT2) 111532260
CJC: 100391738(B3GAT1) 100395367(B3GAT3) 100401353(B3GAT2)
SBQ: 101029437(B3GAT2) 101034661(B3GAT3) 101037144(B3GAT1)
MMUR: 105857314(B3GAT1) 105863337(B3GAT2) 105878602(B3GAT3)
MMU: 280645(B3gat2) 72727(B3gat3) 76898(B3gat1)
MCAL: 110285876(B3gat3) 110294650(B3gat2) 110301609(B3gat1)
MPAH: 110320357(B3gat3) 110321654(B3gat2) 110327443(B3gat1)
RNO: 117108(B3gat1) 293722(B3gat3) 64544(B3gat2)
MCOC: 116072896(B3gat1) 116088248(B3gat2) 116103532(B3gat3)
MUN: 110555732(B3gat2) 110557462(B3gat3) 110563848(B3gat1)
CGE: 100689419(B3gat3) 100750701(B3gat1) 100756696(B3gat2)
PLEU: 114683612(B3gat3) 114692323(B3gat2) 114695282(B3gat1)
NGI: 103737681(B3gat3) 103750906(B3gat1) 103752492(B3gat2)
HGL: 101709113(B3gat2) 101710890(B3gat1) 101717898(B3gat3)
CPOC: 100715995(B3gat1) 100731926(B3gat3)
CCAN: 109674376(B3gat1) 109681240(B3gat3) 109688753(B3gat2)
OCU: 100345204(B3GAT1) 100349666(B3GAT2) 100350294(B3GAT3)
OPI: 101520526(B3GAT1) 101521347(B3GAT3) 101532510(B3GAT2)
TUP: 102471121 102471547(B3GAT1) 102472891(B3GAT3) 102483923(B3GAT2)
CFA: 481875(B3GAT2) 483785(B3GAT3) 489265(B3GAT1)
VVP: 112911944(B3GAT1) 112914116(B3GAT2) 112924375(B3GAT3)
VLG: 121484206(B3GAT2) 121500454(B3GAT1) 121501700(B3GAT3)
AML: 100467358(B3GAT3) 100468570(B3GAT1) 100474997(B3GAT2)
UMR: 103657469(B3GAT2) 103660755(B3GAT1) 103678187(B3GAT3)
UAH: 113247005(B3GAT3) 113259843(B3GAT1) 113261131(B3GAT2)
ORO: 101371974(B3GAT2) 101373124(B3GAT3) 101374853(B3GAT1)
MPUF: 101672086(B3GAT2) 101672751(B3GAT1) 101693078(B3GAT3)
EJU: 114204520(B3GAT2) 114220493(B3GAT3) 114222346(B3GAT1)
MLX: 118001248(B3GAT2) 118013115(B3GAT1) 118015942(B3GAT3)
FCA: 101087404(B3GAT2) 101089044(B3GAT3) 101096760(B3GAT1)
PYU: 121023675(B3GAT3) 121029653(B3GAT2) 121035664(B3GAT1)
PBG: 122483172(B3GAT1) 122483892(B3GAT3) 122489677(B3GAT2)
PTG: 102948861(B3GAT3) 102956846(B3GAT1)
PPAD: 109246589(B3GAT3) 109248245(B3GAT2) 109250412(B3GAT1)
AJU: 106975157(B3GAT1) 106981737(B3GAT3) 113598808(B3GAT2)
HHV: 120240931(B3GAT2) 120246329(B3GAT1) 120249216(B3GAT3)
BTA: 404162(B3GAT3) 541595(B3GAT2) 541596(B3GAT1)
BOM: 102265083(B3GAT1) 102269297(B3GAT3) 102276804(B3GAT2)
BIU: 109554349(B3GAT3) 109563701(B3GAT2) 109569771(B3GAT1)
BBUB: 102393552(B3GAT2) 102395791(B3GAT1) 102399906(B3GAT3)
CHX: 102172516(B3GAT3) 102174927(B3GAT1) 102180686(B3GAT2)
OAS: 101102248(B3GAT2) 101111917(B3GAT3) 101116508(B3GAT1)
ODA: 120874565(B3GAT3) 120877138(B3GAT1) 120878667(B3GAT2)
CCAD: 122422507(B3GAT2) 122430606(B3GAT3) 122450200(B3GAT1)
SSC: 100520760(B3GAT1) 100526195(B3GAT2) 397642(B3GAT3)
CFR: 102503662(B3GAT2) 102505052(B3GAT1) 102505525(B3GAT3)
CBAI: 105065389(B3GAT1) 105070128(B3GAT3)
CDK: 105085061(B3GAT2) 105091529(B3GAT3) 105104231(B3GAT1)
BACU: 103002629(B3GAT3) 103005848(B3GAT1)
LVE: 103078862(B3GAT3) 103085675(B3GAT1) 103090698(B3GAT2)
OOR: 101270376(B3GAT2) 101272042(B3GAT1) 101279944(B3GAT3)
DLE: 111165475(B3GAT1) 111183261(B3GAT2) 111183870(B3GAT3)
PCAD: 102980809(B3GAT1) 102984482(B3GAT2) 102996092(B3GAT3)
PSIU: 116758116(B3GAT3) 116758132(B3GAT1) 116763789(B3GAT2)
ECB: 100063587(B3GAT3) 100071000(B3GAT2) 100072914(B3GAT1)
EPZ: 103558932(B3GAT3) 103565179(B3GAT1)
EAI: 106826833(B3GAT3) 106834293(B3GAT2) 106846908(B3GAT1)
MYB: 102238806(B3GAT2) 102248586(B3GAT1)
MYD: 102752313(B3GAT3) 102763619(B3GAT2) 102768404(B3GAT1)
MMYO: 118648962(B3GAT3) 118660310(B3GAT2) 118678546(B3GAT1)
MLF: 102427881(B3GAT1) 102440153(B3GAT3) 102443090(B3GAT2)
MNA: 107539435(B3GAT1) 107543104(B3GAT3)
PKL: 118705098(B3GAT2) 118707238(B3GAT3) 118725519(B3GAT1)
HAI: 109374153(B3GAT1) 109384098(B3GAT3) 109394168(B3GAT2)
DRO: 112301851(B3GAT1) 112317373(B3GAT3) 112319343(B3GAT2)
SHON: 118975232(B3GAT1) 118978741(B3GAT2) 118991611(B3GAT3)
AJM: 119035322(B3GAT3) 119059152(B3GAT2) 119059614(B3GAT1)
PDIC: 114494114(B3GAT2) 114499679(B3GAT3) 114509699(B3GAT1)
MMF: 118620943(B3GAT2) 118628796(B3GAT3) 118639957(B3GAT1)
RFQ: 117017403(B3GAT1) 117018491(B3GAT2) 117031229(B3GAT3)
PALE: 102886975(B3GAT2) 102890779(B3GAT1) 102891455(B3GAT3)
PGIG: 120594985(B3GAT2) 120600844(B3GAT3) 120615670(B3GAT1)
RAY: 107501598(B3GAT3) 107516621(B3GAT1) 107517181(B3GAT2)
MJV: 108388893(B3GAT1) 108393625(B3GAT3)
TOD: 119240884(B3GAT1) 119250084(B3GAT3) 119252611(B3GAT2)
LAV: 100653777(B3GAT1) 100661019(B3GAT2) 100672369(B3GAT3)
MDO: 100010236(B3GAT3) 100016092(B3GAT2) 100017866(B3GAT1)
GAS: 123240618(B3GAT1) 123245511(B3GAT2) 123251159(B3GAT3) 123253988 123254274
SHR: 100913671(B3GAT1) 100917776(B3GAT2) 100935255(B3GAT3)
PCW: 110195593(B3GAT3) 110195865(B3GAT1) 110217665(B3GAT2)
OAA: 100075413(B3GAT3) 100090509(B3GAT1) 114809412(B3GAT2)
GGA: 373949(B3GAT1) 408180(B3GAT1L) 428638(B3GAT2)
PCOC: 116233086(B3GAT1) 116240683(B3GAT2) 116242363
MGP: 100548173 100550564(B3GAT1) 100550793(B3GAT2)
CJO: 107312060(B3GAT2) 107320417 107324164(B3GAT1)
NMEL: 110387646(B3GAT1) 110395574(B3GAT2) 110399421
APLA: 101791983 101796136(B3GAT2) 101803867(B3GAT1)
ACYG: 106037591 106040589(B3GAT1)
TGU: 100224397(B3GAT1) 100227480 100230653(B3GAT2)
LSR: 110468176(B3GAT1) 110480705 110483934(B3GAT2)
SCAN: 103812436 103822647(B3GAT2) 103823633(B3GAT1)
PMOA: 120506413 120508372(B3GAT1) 120509425 120512443(B3GAT2)
OTC: 121339074 121339125(B3GAT2) 121339265 121340849(B3GAT1)
PRUF: 121351711 121358881(B3GAT2) 121360616(B3GAT1)
GFR: 102034447 102039444(B3GAT1)
FAB: 101805893(B3GAT1) 101808720(B3GAT2) 101809294
PHI: 102105592(B3GAT3) 102107435(B3GAT1) 102108514 102109761(B3GAT2)
PMAJ: 107201433(B3GAT2) 107212820 107214391(B3GAT1)
CCAE: 111926021(B3GAT2) 111939130 111939336(B3GAT1)
CCW: 104687055 104692121(B3GAT1) 104693116(B3GAT2)
ETL: 114055094 114057640(B3GAT2) 114059530(B3GAT1)
FPG: 101912858(B3GAT1) 101915992
FCH: 102051462 102056555(B3GAT1)
CLV: 102094135 102096632(B3GAT1)
EGZ: 104126495 104129214(B3GAT1)
NNI: 104008641 104016633(B3GAT1) 104022039(B3GAT2)
ACUN: 113480922 113488526(B3GAT1)
PADL: 103921445 103924678(B3GAT2) 103924797(B3GAT1)
AAM: 106490243(B3GAT1) 106490700
AROW: 112960466 112968950(B3GAT1) 112977538(B3GAT2)
NPD: 112947263(B3GAT1) 112951787 112957603(B3GAT2)
DNE: 112980767(B3GAT2) 112982679(B3GAT1) 112989415
ASN: 102369484 102369978(B3GAT1) 102375316(B3GAT3)
AMJ: 102561089(B3GAT2) 102563043(B3GAT3) 102568198(B3GAT1) 102570391
CPOO: 109318025(B3GAT1) 109319260
GGN: 109292266(B3GAT1) 109304732
CMY: 102933583(B3GAT1) 102934293(B3GAT2) 102937420 102941550(B3GAT3)
CPIC: 101940176(B3GAT3) 101946238(B3GAT1) 101949948(B3GAT2) 101952321
TST: 117868620(B3GAT1) 117875189(B3GAT2) 117886859
CABI: 116825979 116835867(B3GAT3) 116837710(B3GAT1) 116838956(B3GAT2)
MRV: 120375761(B3GAT1) 120395589 120400918(B3GAT2) 120409148(B3GAT3)
ACS: 100556963 100565010(b3gat1) 100567109(b3gat3) 100567640(b3gat2)
PVT: 110074027 110075006(B3GAT2) 110078243(B3GAT1) 110081006(B3GAT3)
SUND: 121915744(B3GAT3) 121925297(B3GAT2) 121926539 121934476(B3GAT1)
PBI: 103050021 103050571(B3GAT2) 103057546(B3GAT3) 103062783(B3GAT1)
PMUR: 107287662 107292413(B3GAT3) 107293174(B3GAT1)
TSR: 106544019(B3GAT2) 106549009 106552144(B3GAT1)
PGUT: 117658422(B3GAT3) 117662177 117668228(B3GAT1) 117674099(B3GAT2)
VKO: 123019092(B3GAT2) 123028085(B3GAT1) 123029474 123032606(B3GAT3)
PMUA: 114585924(B3GAT1) 114586454(B3GAT3) 114593863(B3GAT2) 114595811 114595812 114595813
ZVI: 118075696(B3GAT3) 118078071 118082766(B3GAT2) 118085568 118097062(B3GAT1)
GJA: 107106043 107111644(B3GAT2) 107116871(B3GAT1) 107120072(B3GAT3)
XLA: 108696374(b3gat1.L) 108697547(b3gat1.S) 108709276(b3gat1l.S) 108713680 108716933 398597(b3gat3.L) 494776(b3gat1l.L) 733177(b3gat2.S)
XTR: 100037907(b3gat1) 594895(b3gat3) 594896(b3gat1l) 594928(b3gat2)
NPR: 108790591(B3GAT1) 108794145(B3GAT3) 108797175 108798068(B3GAT2) 108803353
DRE: 100332644(b3gat1b) 192334(b3gat3) 445060(b3gat2) 548607(b3gat1a)
IPU: 108263352(b3gat2) 108263969 108268305(b3gat3) 108276863
PHYP: 113526410(b3gat1) 113539858(b3gat3) 113540398(b3gat2) 113544801
AMEX: 103028547(b3gat3) 103033187(b3gat2) 103036350 103039617(b3gat1)
EEE: 113579800(b3gat3) 113580728 113585462(b3gat2) 113589586(b3gat1)
TRU: 654312(b3gat3) 654313(b3gat1) 654316(b3gat2)
LCO: 104917661(b3gat2) 104918106(b3gat3) 104933912 104940504
CGOB: 115018257(b3gat1) 115019229 115020060(b3gat2) 115023909(b3gat3)
ELY: 117248179(b3gat2) 117259949(b3gat1a) 117265969 117272641(b3gat3)
PLEP: 121943703 121952766 121954540(b3gat2) 121962326(b3gat3)
SLUC: 116042344(b3gat3) 116043840(b3gat1a) 116044133(b3gat2) 116058219
ECRA: 117934656(b3gat3) 117941777(b3gat1a) 117955564 117960406(b3gat2)
PFLV: 114545607(b3gat3) 114552249 114566225 114572333(b3gat2)
GAT: 120815825(b3gat1a) 120820958(b3gat2) 120822187 120822759(b3gat3)
PPUG: 119213797(b3gat2) 119215605 119223329(b3gat3) 119223336(b3gat1a)
MSAM: 119883000(b3gat1a) 119892912 119895935 119912927(b3gat3) 119917316(b3gat2)
CUD: 121504191(b3gat3) 121510602(b3gat2) 121518892 121527024(b3gat1a)
MZE: 101480695(b3gat3) 101481864 101484521(b3gat2) 101487321
OAU: 116317880(b3gat3) 116321331(b3gat2) 116323301(b3gat1a) 116332802 116335539
OLA: 100049460(b3gat3) 100302419(b3gat1) 100302420(b3gat2) 101158332
OML: 112138523 112149137(b3gat1a) 112156302(b3gat3) 112161511(b3gat2)
XMA: 102235338 102237884 102238000(b3gat3) 102238009(b3gat2)
XCO: 114137391(b3gat2) 114153496 114157307(b3gat3) 114161811
XHE: 116713147(b3gat2) 116728837 116733254(b3gat3) 116737430
PRET: 103474772 103476133 103477310(b3gat2) 103480760(b3gat3)
GAF: 122820549(b3gat3) 122832338(b3gat1a) 122842280(b3gat2) 122845020(b3gat1b)
NFU: 107376425(b3gat2) 107377135(b3gat3) 107380714 107386313
KMR: 108238185 108239122(b3gat2) 108242856(b3gat3) 108244718
ALIM: 106515317(b3gat2) 106524984(b3gat3) 106525648(b3gat1)
CSEM: 103377670(b3gat1) 103384246(b3gat3) 103386917(b3gat2) 103394811 103399064
SSEN: 122766531(b3gat3) 122769239 122773430 122779914(b3gat1b) 122782218(b3gat2)
HHIP: 117751990(b3gat3) 117772341(b3gat1a) 117774236 117775142(b3gat2)
LCF: 108888787(b3gat3) 108893453 108895748(b3gat2) 108898828
SDU: 111220566(b3gat2) 111222183 111231304(b3gat3) 111233438
SLAL: 111663549 111665446 111666406(b3gat3) 111669448(b3gat2)
XGL: 120792109(b3gat1a) 120796615(b3gat2) 120797325(b3gat3) 120802616
HCQ: 109512903(b3gat1) 109515067(b3gat3) 109523236(b3gat2)
ELS: 105005687(b3gat3) 105006566 105010636(b3gat2) 105023813
LOC: 102689893(b3gat2) 102692597 102692986(b3gat1)
LCM: 102348408 102350544(B3GAT1) 102364469(B3GAT2) 102364764(B3GAT3)
CMK: 103171591(b3gat3) 103180285 103183513 103185598(b3gat2)
BFO: 118409812
BBEL: 109470262
DME: Dmel_CG32775(GlcAT-I) Dmel_CG3881(GlcAT-S) Dmel_CG6207(GlcAT-P)
DSI: Dsimw501_GD12815(Dsim_GD12815) Dsimw501_GD16673(Dsim_GD16673) Dsimw501_GD23619(Dsim_GD23619)
DYA: Dyak_GE16334(Dyak_GlcAT-I) Dyak_GE18858(Dyak_GlcAT-S) Dyak_GE20229(Dyak_GlcAT-P)
NVI: 100123025(B3gat)
CSOL: 105363217
TPRE: 106654103
APLN: 108735077
OTU: 111414430
BMAN: 114240951
CLEC: 106661064
HHAL: 106679510
CSCU: 111620730
PTEP: 107447707
SDM: 118182299
CEL: CELE_ZK1307.5(sqv-8)
CBR: CBG_01067(Cbr-sqv-8)
BMY: BM_BM7663(Bma-sqv-8)
GAE: 121371236
CRG: 105326288
MYI: 110466531
PMAX: 117323607
OBI: 106873166
OSN: 115217391
LAK: 106166698
HMG: 100200928
DCT: 110104198
 » show all
Reference
1  [PMID:5770003]
  Authors
Helting T, Roden L.
  Title
Biosynthesis of chondroitin sulfate. II. Glucuronosyl transfer in the formation of the carbohydrate-protein linkage region.
  Journal
J Biol Chem 244:2799-805 (1969)
Reference
2  [PMID:4260846]
  Authors
Helting T.
  Title
Biosynthesis of heparin. Solubilization and partial purification of uridine diphosphate glucuronic acid: acceptor glucuronosyltransferase from mouse mastocytoma.
  Journal
J Biol Chem 247:4327-32 (1972)
Reference
3  [PMID:9506957]
  Authors
Kitagawa H, Tone Y, Tamura J, Neumann KW, Ogawa T, Oka S, Kawasaki T, Sugahara K
  Title
Molecular cloning and expression of glucuronyltransferase I involved in the biosynthesis of the glycosaminoglycan-protein linkage region of proteoglycans.
  Journal
J Biol Chem 273:6615-8 (1998)
DOI:10.1074/jbc.273.12.6615
  Sequence
[hsa:26229]
Other DBs
ExplorEnz - The Enzyme Database: 2.4.1.135
IUBMB Enzyme Nomenclature: 2.4.1.135
ExPASy - ENZYME nomenclature database: 2.4.1.135
BRENDA, the Enzyme Database: 2.4.1.135
CAS: 227184-75-0

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