KEGG   ENZYME: 2.4.1.223Help
Entry
EC 2.4.1.223                Enzyme                                 

Name
glucuronyl-galactosyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase;
alpha-N-acetylglucosaminyltransferase I;
alpha1,4-N-acetylglucosaminyltransferase;
glucuronosylgalactosyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase
Class
Transferases;
Glycosyltransferases;
Hexosyltransferases
BRITE hierarchy
Sysname
UDP-N-acetyl-D-glucosamine:beta-D-glucuronosyl-(1->3)-beta-D-galactosyl-(1->3)-beta-D-galactosyl-(1->4)-beta-D-xylosyl-proteoglycan 4IV-alpha-N-acetyl-D-glucosaminyltransferase
Reaction(IUBMB)
UDP-N-acetyl-D-glucosamine + beta-D-glucuronosyl-(1->3)-beta-D-galactosyl-(1->3)-beta-D-galactosyl-(1->4)-beta-D-xylosyl-proteoglycan = UDP + alpha-N-acetyl-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-(1->3)-beta-D-galactosyl-(1->3)-beta-D-galactosyl-(1->4)-beta-D-xylosyl-proteoglycan [RN:R05930]
Reaction(KEGG)
R05930(G);
(other) R07397
Show
Substrate
UDP-N-acetyl-D-glucosamine [CPD:C00043];
beta-D-glucuronosyl-(1->3)-beta-D-galactosyl-(1->3)-beta-D-galactosyl-(1->4)-beta-D-xylosyl-proteoglycan
Product
UDP [CPD:C00015];
alpha-N-acetyl-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-(1->3)-beta-D-galactosyl-(1->3)-beta-D-galactosyl-(1->4)-beta-D-xylosyl-proteoglycan
Comment
Enzyme involved in the initiation of heparin and heparan sulfate synthesis, transferring GlcNAc to the (GlcA-Gal-Gal-Xyl-)Ser core. Apparently products of both the human EXTL2 and EXTL3 genes can catalyse this reaction. In Caenorhabditis elegans, the product of the rib-2 gene displays this activity as well as that of EC 2.4.1.224, glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase. For explanation of the use of a superscript in the systematic name, see 2-Carb-37.2.)
History
EC 2.4.1.223 created 2002
Pathway
Glycosaminoglycan biosynthesis - heparan sulfate / heparin
Metabolic pathways
Orthology
K02369  
alpha-1,4-N-acetylglucosaminyltransferase EXTL2
K02370  
alpha-1,4-N-acetylglucosaminyltransferase EXTL3
Genes
HSA: 
2135(EXTL2) 2137(EXTL3)
PTR: 
457060(EXTL2) 464086(EXTL3)
PPS: 
100977184(EXTL2) 100984535(EXTL3)
GGO: 
101144361(EXTL3) 101153043(EXTL2)
PON: 
100454000(EXTL2) 100458560(EXTL3)
MCC: 
712189(EXTL2) 718543
MCF: 
MMU: 
54616(Extl3) 58193(Extl2)
RNO: 
310803(Extl2) 56819(Extl3)
CGE: 
100751999(Extl3) 100761218(Extl2)
HGL: 
101697847(Extl3) 101726394(Extl2)
TUP: 
102472563(EXTL3) 102478415(EXTL2)
CFA: 
479925(EXTL2) 486091(EXTL3)
AML: 
FCA: 
101089128(EXTL2) 101095491(EXTL3)
PTG: 
102958499(EXTL2) 102961650(EXTL3)
BTA: 
540527(EXTL2) 783970(EXTL3)
BOM: 
PHD: 
102326761(EXTL3) 102336876(EXTL2)
CHX: 
102174617(EXTL2) 102178172(EXTL3)
SSC: 
100152703(EXTL3) 100155305(EXTL2)
CFR: 
102507432(EXTL3) 102518222(EXTL2)
BACU: 
103001155(EXTL2) 103009480(EXTL3)
LVE: 
103078702(EXTL3) 103089228(EXTL2)
ECB: 
100050181(EXTL2) 100054778(EXTL3)
MYB: 
102249572(EXTL2) 102250952(EXTL3)
MYD: 
102754802(EXTL2) 102764127(EXTL3)
PALE: 
102883756(EXTL2) 102889624(EXTL3)
MDO: 
100030828(EXTL3) 100032966(EXTL2)
SHR: 
100914736(EXTL2) 100919560(EXTL3)
OAA: 
GGA: 
422022(EXTL3) 424465(EXTL2)
MGP: 
TGU: 
100228076(EXTL3) 100232258(EXTL2)
FAB: 
101818645(EXTL3) 101820943(EXTL2)
PHI: 
102105383(EXTL3) 102114416(EXTL2)
APLA: 
101791181(EXTL3) 101792118(EXTL2)
FPG: 
101917228(EXTL3) 101920946(EXTL2)
FCH: 
102049911(EXTL2) 102056733(EXTL3)
CLV: 
102085730(EXTL2) 102088676(EXTL3)
ASN: 
102377426(EXTL3) 102388723(EXTL2)
AMJ: 
102573600(EXTL2) 102575775(EXTL3)
PSS: 
CMY: 
102930795(EXTL2) 102945736(EXTL3)
ACS: 
PBI: 
103050271(EXTL2) 103060383(EXTL3)
XLA: 
443929(extl2) 447542(extl3)
XTR: 
493327(extl2) 493398(extl3)
DRE: 
493783(extl3) 558373(extl2)
TRU: 
MZE: 
OLA: 
XMA: 
LCM: 
102348020(EXTL2) 102361988(EXTL3)
CMK: 
103188516(extl3) 103188850(extl2)
BFO: 
CIN: 
SPU: 
585810(extl3)
DME: 
DPO: 
DAN: 
DER: 
DPE: 
DSE: 
DSI: 
DWI: 
DYA: 
DGR: 
DMO: 
DVI: 
AGA: 
AAG: 
CQU: 
NVI: 
TCA: 
BMOR: 
API: 
PHU: 
ISC: 
CEL: 
CBR: 
CBG18318(Cbr-rib-2)
BMY: 
TSP: 
SMM: 
NVE: 
HMG: 
TAD: 
AQU: 
PVU: 
MTR: 
SBI: 
SORBI_07g027170(SORBIDRAFT_07g027170)
ATR: 
s00025p00194100(AMTR_s00025p00194100)
SMO: 
PPP: 
CCP: 
FGR: 
NHE: 
TRE: 
MAW: 
MAJ: 
CMT: 
EHX: 
TVA: 
 » show all
Taxonomy
Reference
1  [PMID:10318803]
  Authors
Kitagawa H, Shimakawa H, Sugahara K.
  Title
The tumor suppressor EXT-like gene EXTL2 encodes an alpha1, 4-N-acetylhexosaminyltransferase that transfers N-acetylgalactosamine and N-acetylglucosamine to the common glycosaminoglycan-protein linkage region. The key enzyme for the chain initiation of heparan sulfate.
  Journal
J. Biol. Chem. 274 (1999) 13933-7.
  Organism
Homo sapiens
  Sequence
[hsa:2135]
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.
  Organism
Caenorhabditis elegans
  Sequence
Other DBs
ExplorEnz - The Enzyme Database: 
IUBMB Enzyme Nomenclature: 
ExPASy - ENZYME nomenclature database: 
BRENDA, the Enzyme Database: 
CAS: 
179241-74-8

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