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.
History
EC 2.4.1.225 created 2002
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)
MCF: 
MMU: 
14042(Ext1) 14043(Ext2)
RNO: 
299907(Ext1) 311215(Ext2)
CGE: 
100689334(Ext1) 100751585(Ext2)
HGL: 
101706257(Ext1) 101715817(Ext2)
TUP: 
102467803(EXT1) 102487057(EXT2)
CFA: 
475989(EXT2) 482024(EXT1)
AML: 
FCA: 
101081362(EXT1) 101083620(EXT2)
PTG: 
102964333(EXT2) 102968916(EXT1)
BTA: 
281151(EXT2) 538602(EXT1)
BOM: 
102275740(EXT2) 102280308(EXT1)
PHD: 
102315584(EXT2) 102335726(EXT1)
CHX: 
102182243(EXT2) 102182809(EXT1)
SSC: 
100157655(EXT1) 100522362(EXT2)
CFR: 
102508456(EXT1) 102514645(EXT2)
ECB: 
100055879(EXT2) 100056983(EXT1)
MYB: 
102238781(EXT2) 102244714(EXT1)
MYD: 
102765321(EXT1) 102767712(EXT2)
PALE: 
MDO: 
100010947(EXT2) 100025396(EXT1)
SHR: 
100928320(EXT2) 100929895(EXT1)
OAA: 
GGA: 
100858205 420283(EXT1) 425859(EXT2)
MGP: 
TGU: 
100227900(EXT1) 100231605(EXT2)
FAB: 
PHI: 
APLA: 
FPG: 
FCH: 
CLV: 
ASN: 
PSS: 
102444477(EXT1) 102446763(EXT2)
CMY: 
ACS: 
XLA: 
380140(ext2) 398750 399115(ext1)
XTR: 
100151709(ext2) 448393(ext1)
DRE: 
493780(ext2) 497279(ext1a) 497280(ext1b) 497281(ext1c)
TRU: 
MZE: 
OLA: 
XMA: 
LCM: 
BFO: 
CIN: 
SPU: 
DME: 
DPO: 
DAN: 
DER: 
DPE: 
DSE: 
DSI: 
DWI: 
DYA: 
DGR: 
DMO: 
DVI: 
AGA: 
AAG: 
CQU: 
AME: 
408293(ttv) 726216(Ext2)
NVI: 
TCA: 
BMOR: 
API: 
PHU: 
ISC: 
CEL: 
CBR: 
CBG06125(Cbr-rib-1)
BMY: 
LOA: 
TSP: 
SMM: 
NVE: 
HMG: 
TAD: 
AQU: 
GMX: 
FVE: 
BDI: 
SMO: 
PPP: 
CRE: 
GSL: 
ACAN: 
PTI: 
TPS: 
 » 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
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
  Sequence
[bta:281151]
Other DBs
ExplorEnz - The Enzyme Database: 
IUBMB Enzyme Nomenclature: 
ExPASy - ENZYME nomenclature database: 
BRENDA, the Enzyme Database: 
CAS: 
145539-84-0

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