KEGG   ENZYME: 2.4.1.225Help
Entry
EC 2.4.1.225                Enzyme                                 
Name
N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase;
N-acetylglucosaminylproteoglycan beta-1,4-glucuronyltransferase;
heparan glucuronyltransferase II
Class
Transferases;
Glycosyltransferases;
Hexosyltransferases
BRITE hierarchy
Sysname
UDP-alpha-D-glucuronate:N-acetyl-alpha-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-proteoglycan 4-beta-glucuronosyltransferase
Reaction(IUBMB)
UDP-alpha-D-glucuronate + N-acetyl-alpha-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-proteoglycan = UDP + beta-D-glucuronosyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-proteoglycan [RN:R07335]
Reaction(KEGG)
Substrate
UDP-alpha-D-glucuronate [CPD:C00167];
N-acetyl-alpha-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-proteoglycan
Product
UDP [CPD:C00015];
beta-D-glucuronosyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-proteoglycan
Comment
Involved in the biosynthesis of heparin and heparan sulfate. Some forms of the human enzyme (particularly the enzyme complex encoded by the EXT1 and EXT2 genes) act as bifunctional glycosyltransferases, which also have the glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase (EC 2.4.1.224) activity required for the synthesis of the heparan sulfate disaccharide repeats.
Pathway
Glycosaminoglycan biosynthesis - heparan sulfate / heparin
Metabolic pathways
Orthology
K02366  
glucuronyl/N-acetylglucosaminyl transferase EXT1
K02367  
glucuronyl/N-acetylglucosaminyl transferase EXT2
Genes
HSA: 
2131(EXT1) 2132(EXT2)
PTR: 
451140(EXT2) 464349(EXT1)
PPS: 
100975712(EXT2) 100985695(EXT1)
GGO: 
101130563(EXT2) 101133078(EXT1)
PON: 
100172450(EXT1) 100436160(EXT2)
MCC: 
701488(EXT1) 715975(EXT2)
MMU: 
14042(Ext1) 14043(Ext2)
RNO: 
299907(Ext1) 311215(Ext2)
CFA: 
475989(EXT2) 482024(EXT1)
AML: 
FCA: 
101081362(EXT1) 101083620(EXT2)
BTA: 
281151(EXT2) 538602(EXT1)
SSC: 
100157655 100522362
ECB: 
100055879(EXT2) 100056983(EXT1)
MDO: 
100010947(EXT2) 100025396(EXT1)
SHR: 
100928320(EXT2) 100929895(EXT1)
OAA: 
100078206(EXT1) 100079286(EXT2) 100079547 100093607
GGA: 
100858205 420283(EXT1) 425859(EXT2)
MGP: 
100542119 100545109(EXT2) 100551486
TGU: 
100227900(EXT1) 100231605(EXT2)
ACS: 
100553800 100555771(ext2) 100567014
XLA: 
380140(ext2) 398750 399115(ext1)
XTR: 
100151709(ext2) 448393(ext1)
DRE: 
493780(ext2) 497279(ext1a) 497280(ext1b) 497281(ext1c)
TRU: 
101066048 101075972
OLA: 
101163398 101164181 101167217 101169374
BFO: 
BRAFLDRAFT_198850 BRAFLDRAFT_64151(Slc6a15)
CIN: 
100176235 100185120
SPU: 
100893938 577995
DME: 
Dmel_CG10117(ttv) Dmel_CG8433(Ext2)
DPO: 
Dpse_GA10084 Dpse_GA17244
DAN: 
Dana_GF13049 Dana_GF13249
DER: 
Dere_GG20465 Dere_GG22301
DPE: 
Dper_GL11396 Dper_GL11446 Dper_GL16355
DSE: 
Dsec_GM20090 Dsec_GM21554
DSI: 
Dsim_GD11060 Dsim_GD25567
DWI: 
Dwil_GK15814 Dwil_GK15839
DYA: 
Dyak_GE13596 Dyak_GE14097
DGR: 
Dgri_GH20211 Dgri_GH20787
DMO: 
Dmoj_GI20737 Dmoj_GI20872
DVI: 
Dvir_GJ20474 Dvir_GJ20607
AGA: 
AgaP_AGAP000081 AgaP_AGAP010388
AAG: 
AaeL_AAEL003533 AaeL_AAEL005626
CQU: 
CpipJ_CPIJ009765 CpipJ_CPIJ012734
AME: 
408293(ttv) 726216(Ext2)
NVI: 
100120177(NV15289)
TCA: 
657388 659287
API: 
100164685
PHU: 
Phum_PHUM455050 Phum_PHUM488440
ISC: 
IscW_ISCW000326 IscW_ISCW021389 IscW_ISCW021392
CEL: 
CELE_F12F6.3(rib-1)
CBR: 
CBG06125(Cbr-rib-1)
BMY: 
Bm1_15685 Bm1_26565
TSP: 
Tsp_04346
SMM: 
Smp_172060
NVE: 
NEMVE_v1g168451 NEMVE_v1g173450
HMG: 
100208615 100212044
TAD: 
TRIADDRAFT_1026 TRIADDRAFT_24279
AQU: 
100636774 100641175
SMO: 
SELMODRAFT_23945 SELMODRAFT_24030
PTI: 
PHATRDRAFT_11259
TPS: 
THAPSDRAFT_263396
 » show all
Taxonomy
Reference
1  [PMID:11256613]
  Authors
Senay C, Lind T, Muguruma K, Tone Y, Kitagawa H, Sugahara K, Lidholt K, Lindahl U, Kusche-Gullberg M.
  Title
The EXT1/EXT2 tumor suppressors: catalytic activities and role in heparan sulfate biosynthesis.
  Journal
EMBO. Rep. 1 (2000) 282-6.
  Organism
Mus musculus [GN:mmu]
Reference
2  [PMID:9756849]
  Authors
Lind T, Tufaro F, McCormick C, Lindahl U, Lidholt K.
  Title
The putative tumor suppressors EXT1 and EXT2 are glycosyltransferases required for the biosynthesis of heparan sulfate.
  Journal
J. Biol. Chem. 273 (1998) 26265-8.
  Organism
Bos taurus [GN:bta]
Other DBs
ExplorEnz - The Enzyme Database: 
IUBMB Enzyme Nomenclature: 
ExPASy - ENZYME nomenclature database: 
BRENDA, the Enzyme Database: 
CAS: 
145539-84-0
DBGET integrated database retrieval system