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+.
History
EC 2.4.1.135 created 1984, modified 2002
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
MCF: 
101867087 102118326(B3GAT2) 102142387(B3GAT1)
MMU: 
280645(B3gat2) 72727(B3gat3) 76898(B3gat1)
RNO: 
117108(B3gat1) 293722(B3gat3) 64544(B3gat2)
CGE: 
100689419(B3gat3) 100750701(B3gat1) 100756696(B3gat2)
HGL: 
101709113(B3gat2) 101710890(B3gat1) 101717898(B3gat3)
TUP: 
102471121 102471547(B3GAT1) 102472891(B3GAT3) 102483923(B3GAT2)
CFA: 
481875(B3GAT2) 483785(B3GAT3) 489265(B3GAT1)
AML: 
FCA: 
101087404(B3GAT2) 101089044(B3GAT3) 101096760(B3GAT1)
PTG: 
102948861(B3GAT3) 102956846(B3GAT1) 102970934(B3GAT2)
BTA: 
404162(B3GAT3) 541595(B3GAT2) 541596(B3GAT1)
BOM: 
102265083(B3GAT1) 102269297(B3GAT3) 102276804(B3GAT2)
PHD: 
102318873(B3GAT2) 102320142(B3GAT1) 102342113(B3GAT3)
CHX: 
102172516(B3GAT3) 102174927(B3GAT1) 102180686(B3GAT2)
OAS: 
101102248(B3GAT2) 101111917(B3GAT3) 101116508(B3GAT1)
SSC: 
100520760(B3GAT1) 100526195(B3GAT2) 397642(B3GAT3)
CFR: 
102503662(B3GAT2) 102505052(B3GAT1) 102505525(B3GAT3)
BACU: 
103002629(B3GAT3) 103005848(B3GAT1) 103012910(B3GAT2)
LVE: 
103078862(B3GAT3) 103085675(B3GAT1) 103090698(B3GAT2)
ECB: 
100063587(B3GAT3) 100071000(B3GAT2) 100072914(B3GAT1)
MYB: 
102238806 102248586(B3GAT1) 102260939(B3GAT3)
MYD: 
102752313(B3GAT3) 102763619(B3GAT2) 102768404(B3GAT1)
PALE: 
102886975(B3GAT2) 102890779(B3GAT1) 102891455(B3GAT3)
MDO: 
100010236(B3GAT3) 100016092(B3GAT2) 100017866(B3GAT1)
SHR: 
100913671(B3GAT1) 100917776(B3GAT2) 100935255(B3GAT3)
OAA: 
GGA: 
373949(B3GAT1) 408180 428638(B3GAT2)
MGP: 
TGU: 
100224397(B3GAT1) 100227480 100230653(B3GAT2)
FAB: 
101805893(B3GAT1) 101808720(B3GAT2) 101809294
PHI: 
102105592(B3GAT3) 102107435(B3GAT1) 102108514 102109761(B3GAT2)
APLA: 
FPG: 
101912858(B3GAT1) 101915992 101921056(B3GAT2)
FCH: 
102051462 102056555(B3GAT1) 102060053(B3GAT2)
CLV: 
ASN: 
102369484 102369978(B3GAT1) 102375316(B3GAT3) 102385987(B3GAT2)
AMJ: 
102561089(B3GAT2) 102563043(B3GAT3) 102568198(B3GAT1) 102570391
PSS: 
102453546 102455157(B3GAT2) 102460502(B3GAT3)
CMY: 
102933583(B3GAT1) 102934293(B3GAT2) 102937420 102941550(B3GAT3)
ACS: 
100556963 100565010(b3gat1) 100567109(b3gat3) 100567640(b3gat2)
PBI: 
103050021 103050571(B3GAT2) 103057546(B3GAT3) 103062783(B3GAT1)
XLA: 
398597(b3gat3) 494776(b3gat1) 733177(b3gat2)
XTR: 
100037907 594895(b3gat3) 594896(b3gat1) 594928(b3gat2)
DRE: 
192334(b3gat3) 445060(b3gat2) 548607(b3gat1a)
TRU: 
654312(b3gat3) 654313(b3gat1) 654316(b3gat2)
MZE: 
OLA: 
100049460(b3gat3) 100302419(b3gat1) 100302420(b3gat2)
XMA: 
LCM: 
CMK: 
103171591(b3gat3) 103180285(b3gat1) 103183513 103185598(b3gat2)
BFO: 
CIN: 
778533(uglcat1)
SPU: 
DME: 
Dmel_CG32775(GlcAT-I) Dmel_CG3881(GlcAT-S) Dmel_CG6207(GlcAT-P)
DPO: 
DAN: 
DER: 
DPE: 
DSE: 
DSI: 
DWI: 
DYA: 
Dyak_GE16334(Dyak_GlcAT-I) Dyak_GE18858(Dyak_GlcAT-S) Dyak_GE20229(Dyak_GlcAT-P)
DGR: 
DMO: 
DVI: 
AGA: 
AAG: 
CQU: 
AME: 
411457(GlcAT-P) 413271(GlcAT-I)
NVI: 
100123025(B3gat)
TCA: 
BMOR: 
API: 
PHU: 
ISC: 
CEL: 
CBR: 
CBG01067(Cbr-sqv-8)
BMY: 
LOA: 
SMM: 
NVE: 
HMG: 
TAD: 
AQU: 
MDM: 
SBI: 
SORBI_03g011010(SORBIDRAFT_03g011010)
ATR: 
s00033p00038120(AMTR_s00033p00038120)
SMO: 
PPP: 
BPG: 
MPP: 
MBR: 
EHX: 
 » 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.
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.
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.
  Sequence
[hsa:26229]
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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