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.)
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
MMU: 
54616(Extl3) 58193(Extl2)
RNO: 
310803(Extl2) 56819(Extl3)
CFA: 
479925(EXTL2) 486091(EXTL3)
AML: 
FCA: 
101089128(EXTL2) 101095491(EXTL3)
BTA: 
540527(EXTL2) 783970(EXTL3)
SSC: 
100152703(EXTL3) 100155305(EXTL2)
ECB: 
100050181 100054778(EXTL3)
MDO: 
100030828(EXTL3) 100032966
SHR: 
100914736(EXTL2) 100919560(EXTL3)
OAA: 
100074116 100087229 100091427
GGA: 
422022(EXTL3) 424465(EXTL2)
MGP: 
100542317(EXTL3) 100549273
TGU: 
100228076(EXTL3) 100232258(EXTL2)
ACS: 
100557117(extl3) 100563405
XLA: 
443929(extl2) 447542(extl3)
XTR: 
493327(extl2) 493398(extl3)
DRE: 
493783(extl3) 558373(extl2)
TRU: 
101066160 101068405
OLA: 
101163453 101174221
BFO: 
BRAFLDRAFT_199038
CIN: 
100184170
SPU: 
585810(extl3)
DME: 
Dmel_CG15110(botv)
DPO: 
Dpse_GA13499
DAN: 
Dana_GF11490
DER: 
Dere_GG21945
DPE: 
Dper_GL11868
DSE: 
Dsec_GM21933
DSI: 
Dsim_GD11429
DWI: 
Dwil_GK17964
DYA: 
Dyak_GE12019
DGR: 
Dgri_GH23150
DMO: 
Dmoj_GI18912
DVI: 
Dvir_GJ19767
AGA: 
AgaP_AGAP001688
AAG: 
AaeL_AAEL000483
CQU: 
CpipJ_CPIJ016867
NVI: 
100121031(NV16740)
TCA: 
663383
API: 
100167784
PHU: 
Phum_PHUM490250
ISC: 
IscW_ISCW011872
CEL: 
CELE_K01G5.6(rib-2)
CBR: 
CBG18318(Cbr-rib-2)
BMY: 
Bm1_27215
TSP: 
Tsp_02636
SMM: 
Smp_073220
NVE: 
NEMVE_v1g235881
HMG: 
100212160
TAD: 
TRIADDRAFT_50846
AQU: 
100633921 100640795
PPP: 
PHYPADRAFT_146391
FGR: 
FG04406.1
NHE: 
NECHADRAFT_45533 NECHADRAFT_84083
TVA: 
TVAG_466230
 » 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 [GN:hsa]
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
Homo sapiens [GN:hsa]
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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