KEGG   ENZYME: 2.4.1.135Help
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
Class
Transferases;
Glycosyltransferases;
Hexosyltransferases
BRITE hierarchy
Sysname
UDP-glucuronate:3-beta-D-galactosyl-4-beta-D-galactosyl-O-beta-D-xylosylprotein D-glucuronosyltransferase
Reaction(IUBMB)
UDP-glucuronate + 3-beta-D-galactosyl-4-beta-D-galactosyl-O-beta-D-xylosylprotein = UDP + 3-beta-D-glucuronosyl-3-beta-D-galactosyl-4-beta-D-galactosyl-O-beta-D-xylosylprotein [RN:R04607 R05928]
Reaction(KEGG)
Substrate
UDP-glucuronate [CPD:C00167];
3-beta-D-galactosyl-4-beta-D-galactosyl-O-beta-D-xylosylprotein [CPD:C04825]
Product
UDP [CPD:C00015];
3-beta-D-glucuronosyl-3-beta-D-galactosyl-4-beta-D-galactosyl-O-beta-D-xylosylprotein [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+.
Pathway
Other types of O-glycan biosynthesis
Glycosaminoglycan biosynthesis - chondroitin sulfate / dermatan sulfate
Glycosaminoglycan biosynthesis - heparan sulfate / heparin
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
Genes
HSA: 
135152(B3GAT2) 26229(B3GAT3) 27087(B3GAT1)
PTR: 
462813(B3GAT2) 466859(B3GAT1) 740388(B3GAT3)
PPS: 
100980067(B3GAT1) 100993761(B3GAT2)
GGO: 
101132051(B3GAT2) 101142201(B3GAT1)
PON: 
100448790(B3GAT1) 100458701(B3GAT3)
MCC: 
695293(B3GAT1) 717157(B3GAT2) 718744
MMU: 
280645(B3gat2) 72727(B3gat3) 76898(B3gat1)
RNO: 
117108(B3gat1) 293722(B3gat3) 64544(B3gat2)
CFA: 
481875(B3GAT2) 483785(B3GAT3) 489265(B3GAT1)
AML: 
FCA: 
101087404(B3GAT2) 101089044(B3GAT3) 101096760(B3GAT1)
BTA: 
404162(B3GAT3) 541595(B3GAT2) 541596(B3GAT1)
SSC: 
100520760(B3GAT1) 100526195 397642(B3GAT3)
ECB: 
100063587 100072914(B3GAT1)
MDO: 
100010236 100016092 100017866(B3GAT1)
SHR: 
100913671(B3GAT1) 100917776(B3GAT2) 100935255(B3GAT3)
OAA: 
100075217 100075413 100090509
GGA: 
373949(B3GAT1) 408180 428638(B3GAT2)
MGP: 
100550564 100550793
TGU: 
100224397(B3GAT1) 100227480 100230653(B3GAT2)
ACS: 
100556963 100565010 100567109 100567640
XLA: 
398597(b3gat3) 494776(b3gat1) 733177(b3gat2)
XTR: 
100037907 594895(b3gat3) 594896(b3gat1) 594928(b3gat2)
DRE: 
192334(b3gat3) 445060(b3gat2) 548607(b3gat1)
TRU: 
654312(b3gat3) 654313(b3gat1) 654316(b3gat2)
OLA: 
100049460(b3gat3) 100302419(b3gat1) 100302420(b3gat2)
BFO: 
BRAFLDRAFT_92974
CIN: 
778533(uglcat1)
SPU: 
579023
DME: 
Dmel_CG32775(GlcAT-I) Dmel_CG3881(GlcAT-S) Dmel_CG6207(GlcAT-P)
DPO: 
Dpse_GA17748 Dpse_GA19440
DAN: 
Dana_GF20780 Dana_GF22782 Dana_GF24562
DER: 
Dere_GG10044 Dere_GG13931 Dere_GG18695
DPE: 
Dper_GL14437 Dper_GL18922 Dper_GL22838
DSE: 
Dsec_GM12329 Dsec_GM17591 Dsec_GM24766
DSI: 
Dsim_GD12815 Dsim_GD16673 Dsim_GD23619
DWI: 
Dwil_GK15595 Dwil_GK20549 Dwil_GK23869
DYA: 
Dyak_GE16334(Dyak_GlcAT-I) Dyak_GE18858(Dyak_GlcAT-S) Dyak_GE20229(Dyak_GlcAT-P)
DGR: 
Dgri_GH10677 Dgri_GH16660 Dgri_GH24148
DMO: 
Dmoj_GI11095 Dmoj_GI11619 Dmoj_GI16402
DVI: 
Dvir_GJ11297 Dvir_GJ17023 Dvir_GJ18316
AGA: 
AgaP_AGAP001367 AgaP_AGAP002801 AgaP_AGAP008806
AAG: 
AaeL_AAEL004974 AaeL_AAEL006254 AaeL_AAEL009174
CQU: 
CpipJ_CPIJ004318 CpipJ_CPIJ007101 CpipJ_CPIJ009270
AME: 
411457(GlcAT-P) 413271(GlcAT-I)
NVI: 
100123025(B3gat)
TCA: 
656067 663511
API: 
100158683 100159562 100161409
PHU: 
Phum_PHUM032300 Phum_PHUM392280
ISC: 
IscW_ISCW006942 IscW_ISCW019389
CEL: 
CELE_ZK1307.5(sqv-8)
CBR: 
CBG01067(Cbr-sqv-8)
BMY: 
Bm1_42955
SMM: 
Smp_083130
NVE: 
NEMVE_v1g98778
HMG: 
100200928
TAD: 
TRIADDRAFT_49638
AQU: 
100632704 100632957
PPP: 
PHYPADRAFT_57016
MBR: 
MONBRDRAFT_16483
 » show all
Taxonomy
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 (1969) 2799-805.
  Organism
Gallus gallus [GN:gga]
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 (1972) 4327-32.
  Organism
Mus musculus [GN:mmu]
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 (1998) 6615-8.
  Organism
Homo sapiens [GN:hsa]
Other DBs
ExplorEnz - The Enzyme Database: 
IUBMB Enzyme Nomenclature: 
ExPASy - ENZYME nomenclature database: 
BRENDA, the Enzyme Database: 
CAS: 
227184-75-0
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