| Entry |
|
|---|
| Name |
N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase;
N-acetylglucosaminylproteoglycan beta-1,4-glucuronyltransferase;
heparan glucuronyltransferase II |
|---|
| Class |
Transferases;
Glycosyltransferases;
Hexosyltransferases
 |
|---|
| 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) |
R07335 > R05935(G)
 |
|---|
| 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 |
PATH: ec00534 Heparan sulfate biosynthesis |
|---|
| Orthology |
KO: K02366 glucuronyl/N-acetylglucosaminyl transferase EXT1
KO: K02367 glucuronyl/N-acetylglucosaminyl transferase EXT2 |
|---|
| Genes |
HSA: 2131(EXT1) 2132(EXT2)
PTR: 451140(EXT2) 464349(EXT1)
MCC: 701488(EXT1) 715975(EXT2)
MMU: 14042(Ext1) 14043(Ext2)
RNO: 299907(Ext1) 311215(Ext2)
CFA: 475989(EXT2) 482024(EXT1)
BTA: 281151(EXT2) 538602(EXT1)
SSC: 100157655
ECB: 100055879 100056983
MDO: 100010947 100025396
OAA: 100078206 100079286 100093607
GGA: 420283(EXT1) 425859(RCJMB04_7p22)
TGU: 100227900 100231605
XLA: 380140(ext2) 398750 399115(xext1)
XTR: 100151709(ext2) 448393(ext1)
DRE: 493780(ext2) 497279(ext1a) 497280(ext1b) 497281(ext1c)
BFO: BRAFLDRAFT_198850 BRAFLDRAFT_64151(Slc6a15)
CIN: 100185120
SPU: 577995
DME: Dmel_CG10117(ttv) Dmel_CG8433(Ext2)
DPO: Dpse_GA10084 Dpse_GA17244
DAN: Dana_GF13049 Dana_GF13249
DER: Dere_GG20465
DPE: Dper_GL11446
DSE: Dsec_GM21554
DSI: Dsim_GD11060
DYA: Dyak_GE13596
DGR: Dgri_GH20787
DMO: Dmoj_GI20872
AGA: AgaP_AGAP000081 AgaP_AGAP010388
AAG: AaeL_AAEL003533 AaeL_AAEL005626
CQU: CpipJ_CPIJ012734
AME: 408293 726216
NVI: 100120177
TCA: 657388 659287
API: 100167795
ISC: IscW_ISCW021392
CEL: F12F6.3(rib-1)
CBR: CBG06125
BMY: Bm1_26565
SMM: Smp_172060
NVE: NEMVE_v1g168451 NEMVE_v1g173450
TAD: TRIADDRAFT_1026
PPP: PHYPADRAFT_123943 PHYPADRAFT_123994
 |
|---|
Reference
Authors
Title
Journal
Organism
|
1 [PMID:11256613]
Senay C, Lind T, Muguruma K, Tone Y, Kitagawa H, Sugahara K, Lidholt
K, Lindahl U, Kusche-Gullberg M.
The EXT1/EXT2 tumor suppressors: catalytic activities and role in
heparan sulfate biosynthesis.
EMBO. Rep. 1 (2000) 282-6.
Mus musculus [GN:mmu] |
|---|
Reference
Authors
Title
Journal
Organism
|
2 [PMID:9756849]
Lind T, Tufaro F, McCormick C, Lindahl U, Lidholt K.
The putative tumor suppressors EXT1 and EXT2 are
glycosyltransferases required for the biosynthesis of heparan
sulfate.
J. Biol. Chem. 273 (1998) 26265-8.
Bos taurus [GN:bta] |
|---|
| Other DBs |
ExplorEnz - The Enzyme Database: 2.4.1.225
IUBMB Enzyme Nomenclature: 2.4.1.225
ExPASy - ENZYME nomenclature database: 2.4.1.225
BRENDA, the Enzyme Database: 2.4.1.225
CAS: 145539-84-0 |
|---|
