KEGG   ENZYME: 2.4.1.223Help
Entry
EC 2.4.1.223                Enzyme                                 

Name
glucuronosyl-galactosyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase;
alpha-N-acetylglucosaminyltransferase I;
alpha1,4-N-acetylglucosaminyltransferase;
glucuronosylgalactosyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase;
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;
glucuronyl-galactosyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase
Class
Transferases;
Glycosyltransferases;
Hexosyltransferases
BRITE hierarchy
Sysname
UDP-N-acetyl-alpha-D-glucosamine:[protein]-3-O-(beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl)-L-serine 4IV-alpha-N-acetyl-D-glucosaminyltransferase (configuration-retaining)
Reaction(IUBMB)
UDP-N-acetyl-alpha-D-glucosamine + [protein]-3-O-(beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl)-L-serine = UDP + [protein]-3-O-(alpha-D-GlcNAc-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl)-L-serine [RN:R05930 R07397]
Reaction(KEGG)
Substrate
UDP-N-acetyl-alpha-D-glucosamine [CPD:C00043];
[protein]-3-O-(beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl)-L-serine [CPD:C04903]
Product
UDP [CPD:C00015];
[protein]-3-O-(alpha-D-GlcNAc-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl)-L-serine [CPD:C15656]
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 {iupac/2carb/37#372::2-Carb-37.2}.
History
EC 2.4.1.223 created 2002, modified 2016
Pathway
ec00534  Glycosaminoglycan biosynthesis - heparan sulfate / heparin
ec01100  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)
NLE: 100585000(EXTL2) 100590557(EXTL3)
MCC: 100426593(EXTL3) 712189(EXTL2)
MCF: 101865433(EXTL3) 102129629(EXTL2)
CSAB: 103215549(EXTL3) 103224361(EXTL2)
RRO: 104668244(EXTL3) 104673259(EXTL2)
RBB: 108524175(EXTL2) 108537365(EXTL3)
CJC: 100400452(EXTL3) 100412156(EXTL2)
SBQ: 101037632(EXTL3) 101043244(EXTL2)
MMU: 54616(Extl3) 58193(Extl2)
MCAL: 110291045(Extl2) 110306109 110309303(Extl3)
MPAH: 110320549(Extl2) 110325330(Extl3)
RNO: 310803(Extl2) 56819(Extl3)
MUN: 110551871(Extl2) 110559132(Extl3)
CGE: 100751999(Extl3) 100761218(Extl2)
NGI: 103729989(Extl3) 103741874(Extl2)
HGL: 101697847(Extl3) 101726394(Extl2)
CCAN: 109682613 109698077(Extl2)
OCU: 100348721(EXTL3) 100352420(EXTL2)
TUP: 102472563(EXTL3) 102478415(EXTL2)
CFA: 479925(EXTL2) 486091(EXTL3)
VVP: 112918475(EXTL2) 112926771(EXTL3)
AML: 100469588(EXTL3) 100480634(EXTL2)
UMR: 103669325(EXTL2) 103675952(EXTL3)
UAH: 113254344(EXTL2) 113269941(EXTL3)
ORO: 101369733(EXTL2) 101377818(EXTL3)
FCA: 101089128(EXTL2) 101095491(EXTL3)
PTG: 102958499(EXTL2) 102961650(EXTL3)
PPAD: 109262363(EXTL3) 109271932(EXTL2)
AJU: 106976417(EXTL2) 106983017(EXTL3)
BTA: 540527(EXTL2) 783970(EXTL3)
BOM: 102265657 102267911(EXTL2) 102273632(EXTL3)
BIU: 109556009(EXTL2) 109562800(EXTL3)
BBUB: 102398616(EXTL3) 102406549(EXTL2)
CHX: 102174617(EXTL2) 102178172(EXTL3)
OAS: 101108814(EXTL3) 101115079(EXTL2)
SSC: 100152703(EXTL3) 100155305(EXTL2)
CFR: 102507432(EXTL3) 102518222(EXTL2)
CDK: 105084916(EXTL2) 105102072(EXTL3)
BACU: 103001155(EXTL2) 103009480(EXTL3)
LVE: 103078702(EXTL3) 103089228(EXTL2)
OOR: 101285385(EXTL2) 101286194(EXTL3)
DLE: 111180111(EXTL2) 111185386(EXTL3)
PCAD: 102987427(EXTL2) 102995934(EXTL3)
ECB: 100050181(EXTL2) 100054778(EXTL3)
EPZ: 103550057(EXTL2) 103567372(EXTL3)
EAI: 106832529(EXTL3) 106837773(EXTL2)
MYB: 102249572(EXTL2) 102250952(EXTL3)
MYD: 102754802(EXTL2) 102764127(EXTL3)
MNA: 107530682(EXTL2) 107537770(EXTL3)
HAI: 109371361(EXTL2) 109379533(EXTL3)
DRO: 112312602(EXTL3) 112314076(EXTL2)
PALE: 102883756(EXTL2) 102889624(EXTL3)
RAY: 107504389(EXTL2) 107510781(EXTL3)
MJV: 108391555(EXTL3) 108407604(EXTL2)
LAV: 100658953(EXTL2) 100666182(EXTL3)
MDO: 100030828(EXTL3) 100032966(EXTL2)
SHR: 100914736(EXTL2) 100919560(EXTL3)
PCW: 110209977(EXTL3) 110217549(EXTL2)
OAA: 100087229(EXTL3) 100091427(EXTL2)
GGA: 422022(EXTL3) 424465(EXTL2)
MGP: 100542317(EXTL3) 100549273(EXTL2)
CJO: 107312356(EXTL3) 107317515(EXTL2)
NMEL: 110396622(EXTL3) 110402398(EXTL2)
APLA: 101791181(EXTL3) 101792118(EXTL2)
ACYG: 106038151(EXTL3) 106039416(EXTL2)
TGU: 100228076(EXTL3) 100232258(EXTL2)
LSR: 110476418(EXTL3) 110477488(EXTL2)
SCAN: 103814860 103821407(EXTL3)
GFR: 102041533(EXTL3)
FAB: 101818645(EXTL3)
PHI: 102105383(EXTL3) 102114416(EXTL2)
PMAJ: 107201996(EXTL3) 107208067(EXTL2)
CCAE: 111927356(EXTL3) 111932725(EXTL2)
CCW: 104696886(EXTL3) 109143175(EXTL2)
ETL: 114061647(EXTL2) 114068426(EXTL3)
FPG: 101917228(EXTL3) 101920946(EXTL2)
FCH: 102049911(EXTL2) 102056733(EXTL3)
CLV: 102085730(EXTL2) 102088676(EXTL3)
EGZ: 104127251(EXTL3)
NNI: 104014701(EXTL3) 104015519(EXTL2)
ACUN: 113478245(EXTL3) 113482992(EXTL2)
PADL: 103913240(EXTL3)
AAM: 106488030(EXTL3) 106499747(EXTL2)
ASN: 102377426(EXTL3) 102388723(EXTL2)
AMJ: 102573600(EXTL2) 102575775(EXTL3)
PSS: 102451232 102462577(EXTL3) 102462903(EXTL2)
CMY: 102930795(EXTL2) 102945736(EXTL3)
CPIC: 101935250(EXTL3) 101945022(EXTL2)
ACS: 100557117(extl3) 100563405(extl2)
PVT: 110070203(EXTL3) 110076514(EXTL2)
PBI: 103050271(EXTL2) 103060383(EXTL3)
PMUR: 107284865(EXTL3) 107302497(EXTL2)
TSR: 106541899(EXTL3) 106549044(EXTL2)
PMUA: 114594927(EXTL3) 114598702(EXTL2)
GJA: 107106370(EXTL3) 107107448(EXTL2)
XLA: 443929(extl2.L) 444275 447542(extl3.L)
XTR: 493327(extl2) 493398(extl3)
NPR: 108791470(EXTL2) 108802201(EXTL3)
DRE: 493783(extl3) 558373(extl2)
IPU: 108257719(extl3)
PHYP: 113532052(extl3)
AMEX: 103035439(extl2) 103041905(extl3)
EEE: 113571699(extl3) 113584240(extl2)
TRU: 101066160(extl2) 101068405(extl3)
LCO: 104934376(extl3) 104936574(extl2)
NCC: 104946504(extl3) 104951471(extl2)
MZE: 101467370(extl3) 101469432(extl2)
ONL: 100694061(extl3) 100711437(extl2)
OLA: 101163453(extl3) 101174221(extl2)
XMA: 102236630(extl2) 102237533(extl3)
XCO: 114146128(extl2) 114158236(extl3)
PRET: 103457379(extl3) 103479408(extl2)
CVG: 107081098(extl3) 107083757(extl2)
NFU: 107377723(extl3) 107384011(extl2)
KMR: 108233500(extl3) 108239591(extl2)
ALIM: 106525671(extl3) 106535008(extl2)
AOCE: 111572767(extl2) 111583150(extl3)
CSEM: 103381243(extl3) 103396080(extl2)
POV: 109636091(extl3) 109640876(extl2)
LCF: 108873476(extl3) 108883513(extl2)
SDU: 111229104(extl2) 111236334(extl3)
SLAL: 111654468(extl2) 111661394(extl3)
HCQ: 109514670(extl3)
BPEC: 110158086(extl2) 110164237(extl3)
MALB: 109957690(extl2) 109962352(extl3)
ELS: 105007545(extl2) 105017562(extl3)
SFM: 108924136(extl3) 108935058(extl2)
PKI: 111841187(extl3)
LCM: 102348020(EXTL2) 102361988(EXTL3)
CMK: 103188516(extl3) 103188850(extl2)
RTP: 109926989(extl3) 109936576(extl2)
CIN: 100184170
SPU: 585810
SKO: 100369934
DME: Dmel_CG15110(botv)
DER: 6547292
DSI: Dsimw501_GD11429(Dsim_GD11429)
DSR: 110184038
DMN: 108160133
DWI: 6646203
DAZ: 108616969
DNV: 108653682
DHE: 111594305
MDE: 101892286
LCQ: 111688369
AAG: 5578446
AALB: 109398955
BIM: 105680701
BTER: 105666123
CCAL: 108622898
OBB: 114871977
SOC: 105200907
MPHA: 105838726
AEC: 105154628
ACEP: 105620069
PBAR: 105433557
VEM: 105564962
HST: 105188784
DQU: 106745718
CFO: 105251656
LHU: 105674049
PGC: 109853827
OBO: 105288161
PCF: 106786799
NVI: 100121031
CSOL: 105365204
MDL: 103569026
TCA: 663383
DPA: 109537502
ATD: 109596360
NVL: 108567151
BMOR: 101742984
BMAN: 114248987
PMAC: 106712471
PRAP: 111000541
HAW: 110370155
TNL: 113493550
PXY: 105388581
API: 100167784
DNX: 107163206
AGS: 114122118
RMD: 113556372
BTAB: 109030556
CLEC: 106672266
ZNE: 110831980
FCD: 110846056
PVM: 113819647
TUT: 107366477
DPTE: 113795874
CSCU: 111639700
PTEP: 107455291
CEL: CELE_K01G5.6(rib-2)
CBR: CBG18318(Cbr-rib-2)
BMY: Bm1_27215
TSP: Tsp_02636
PCAN: 112562383
MYI: 110442614
LAK: 106152057
EPA: 110234330
ADF: 107341811
AMIL: 114970312
PDAM: 113677366
SPIS: 111325684
DGT: 114527489
HMG: 100212160
EGR: 104424321
CMO: 103501448
PSOM: 113354121
SMO: SELMODRAFT_133103(GT64C1-1) SELMODRAFT_133161(GT64C1-2)
PPP: 112292971
MAW: MAC_06017
MAJ: MAA_06147
 » 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 274:13933-7 (1999)
DOI:10.1074/jbc.274.20.13933
  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 276:4834-8 (2001)
DOI:10.1074/jbc.C000835200
  Sequence
Other DBs
ExplorEnz - The Enzyme Database: 2.4.1.223
IUBMB Enzyme Nomenclature: 2.4.1.223
ExPASy - ENZYME nomenclature database: 2.4.1.223
BRENDA, the Enzyme Database: 2.4.1.223
CAS: 179241-74-8

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