KEGG   ENZYME: 2.4.1.224Help
Entry
EC 2.4.1.224                Enzyme                                 

Name
glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase;
alpha-N-acetylglucosaminyltransferase II glucuronyl-N-acetylglucosaminylproteoglycan alpha-1,4-N-acetylglucosaminyltransferase
Class
Transferases;
Glycosyltransferases;
Hexosyltransferases
BRITE hierarchy
Sysname
UDP-N-acetyl-D-glucosamine:beta-D-glucuronosyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase
Reaction(IUBMB)
UDP-N-acetyl-D-glucosamine + beta-D-glucuronosyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-proteoglycan = UDP + N-acetyl-alpha-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-proteoglycan [RN:R07334]
Reaction(KEGG)
Substrate
UDP-N-acetyl-D-glucosamine [CPD:C00043];
beta-D-glucuronosyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-proteoglycan
Product
UDP [CPD:C00015];
N-acetyl-alpha-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-proteoglycan
Comment
Involved in the biosynthesis of heparin and heparan sulfate. Some forms of the enzyme from human (particularly the enzyme complex encoded by the EXT1 and EXT2 genes) act as bifunctional glycosyltransferases, which also have the 4-beta-glucuronosyltransferase (EC 2.4.1.225, N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase) activity required for the synthesis of the heparan sulfate disaccharide repeats. Other human forms of this enzyme (e.g. the product of the EXTL1 gene) have only the 4-alpha-N-acetylglucosaminyltransferase activity. In Caenorhabditis elegans, the product of the rib-2 gene displays the activities of this enzyme as well as EC 2.4.1.223, glucuronyl-galactosyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase.
History
EC 2.4.1.224 created 2002
Pathway
Glycosaminoglycan biosynthesis - heparan sulfate / heparin
Metabolic pathways
Orthology
K02366  
glucuronyl/N-acetylglucosaminyl transferase EXT1
K02367  
glucuronyl/N-acetylglucosaminyl transferase EXT2
K02368  
alpha-1,4-N-acetylglucosaminyltransferase EXTL1
K02370  
alpha-1,4-N-acetylglucosaminyltransferase EXTL3
Genes
HSA: 
2131(EXT1) 2132(EXT2) 2134(EXTL1) 2137(EXTL3)
PTR: 
451140(EXT2) 464086(EXTL3) 464349(EXT1) 469238(EXTL1)
PPS: 
100975712(EXT2) 100979888(EXTL1) 100984535(EXTL3) 100985695(EXT1)
GGO: 
101130563(EXT2) 101133078(EXT1) 101144361(EXTL3) 101149310(EXTL1)
PON: 
100172231(EXTL1) 100172450(EXT1) 100436160(EXT2) 100458560(EXTL3)
MCC: 
701488(EXT1) 713664(EXTL1) 715975(EXT2) 718543
MCF: 
MMU: 
14042(Ext1) 14043(Ext2) 54616(Extl3) 56219(Extl1)
RNO: 
299907(Ext1) 311215(Ext2) 313610(Extl1) 56819(Extl3)
CGE: 
100689334(Ext1) 100751585(Ext2) 100751999(Extl3) 100770000(Extl1)
HGL: 
101697847(Extl3) 101705292(Extl1) 101706257(Ext1) 101715817(Ext2)
TUP: 
102467803(EXT1) 102472563(EXTL3) 102487057(EXT2) 102499532(EXTL1)
CFA: 
475989(EXT2) 482024(EXT1) 486091(EXTL3) 487363(EXTL1)
AML: 
FCA: 
101081362(EXT1) 101083620(EXT2) 101083693(EXTL1) 101095491(EXTL3)
PTG: 
102961650(EXTL3) 102962317(EXTL1) 102964333(EXT2) 102968916(EXT1)
BTA: 
281151(EXT2) 511060(EXTL1) 538602(EXT1) 783970(EXTL3)
BOM: 
102265657 102267135(EXTL1) 102273632(EXTL3) 102275740(EXT2) 102280308(EXT1)
PHD: 
102315584(EXT2) 102326761(EXTL3) 102333864(EXTL1) 102335726(EXT1)
CHX: 
102178172(EXTL3) 102182004(EXTL1) 102182243(EXT2) 102182809(EXT1)
OAS: 
101108814(EXTL3) 101110867(EXT2) 101113394(EXTL1) 101114877(EXT1)
SSC: 
100152703(EXTL3) 100157655(EXT1) 100522362(EXT2) 100623848(EXTL1)
CFR: 
102507432(EXTL3) 102508456(EXT1) 102514645(EXT2) 102521830(EXTL1)
BACU: 
102999728(EXT2) 103004222(EXTL1) 103009480(EXTL3) 103019432(EXT1)
LVE: 
103068452(EXTL1) 103078702(EXTL3) 103078738(EXT1) 103091009(EXT2)
ECB: 
100054778(EXTL3) 100055879(EXT2) 100056983(EXT1) 100071242(EXTL1)
MYB: 
102238781(EXT2) 102244714(EXT1) 102246778(EXTL1) 102250952(EXTL3)
MYD: 
102761567(EXTL1) 102764127(EXTL3) 102765321(EXT1) 102767712(EXT2)
PALE: 
MDO: 
100010947(EXT2) 100025396(EXT1) 100030828(EXTL3)
SHR: 
100918977(EXTL1) 100919560(EXTL3) 100928320(EXT2) 100929895(EXT1)
OAA: 
GGA: 
100858205 420283(EXT1) 422022(EXTL3) 425859(EXT2)
MGP: 
APLA: 
TGU: 
100227900(EXT1) 100228076(EXTL3) 100231605(EXT2)
FAB: 
PHI: 
FPG: 
FCH: 
CLV: 
ASN: 
AMJ: 
PSS: 
CMY: 
ACS: 
100553800(extl1) 100555771(ext2) 100557117(extl3) 100567014(ext1)
PBI: 
XLA: 
380140(ext2) 398750 399115(ext1) 447542(extl3)
XTR: 
100151709(ext2) 448393(ext1) 493398(extl3)
DRE: 
493780(ext2) 493783(extl3) 497279(ext1a) 497280(ext1b) 497281(ext1c)
TRU: 
MZE: 
OLA: 
XMA: 
LCM: 
CMK: 
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) CBG18318(Cbr-rib-2)
BMY: 
LOA: 
TSP: 
SMM: 
NVE: 
HMG: 
TAD: 
AQU: 
GMX: 
PVU: 
MTR: 
FVE: 
PMUM: 
MDM: 
CMO: 
BDI: 
ATR: 
s00025p00194100(AMTR_s00025p00194100)
SMO: 
PPP: 
CRE: 
BPG: 
GSL: 
CCP: 
FGR: 
NHE: 
TRE: 
MAW: 
MAJ: 
CMT: 
ACAN: 
PTI: 
TPS: 
TVA: 
 » show all
Taxonomy
Reference
1  [PMID:11390981]
  Authors
Kim BT, Kitagawa H, Tamura  J, Saito T, Kusche-Gullberg M, Lindahl U, Sugahara K.
  Title
Human tumor suppressor EXT gene family members EXTL1 and EXTL3 encode alpha 1,4- N-acetylglucosaminyltransferases that likely are involved in heparan sulfate/ heparin biosynthesis.
  Journal
Proc. Natl. Acad. Sci. U. S. A. 98 (2001) 7176-81.
Reference
2  [PMID:11121397]
  Authors
Kitagawa H, Egusa N, Tamura JI, Kusche-Gullberg M, Lindahl U, Sugahara K.
  Title
rib-2, a Caenorhabditis elegans homolog of the human tumor suppressor EXT genes encodes a novel alpha1,4-N-acetylglucosaminyltransferase involved in the biosynthetic initiation and elongation of heparan sulfate.
  Journal
J. Biol. Chem. 276 (2001) 4834-8.
  Sequence
Reference
3  [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.
Reference
4  [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.
  Sequence
[bta:281151]
Other DBs
ExplorEnz - The Enzyme Database: 
IUBMB Enzyme Nomenclature: 
ExPASy - ENZYME nomenclature database: 
BRENDA, the Enzyme Database: 
CAS: 
336193-98-7

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