KEGG   ENZYME: 2.4.1.223
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
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)
R05930(G) R07397
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
HSA2135(EXTL2) 2137(EXTL3)
PTR457060(EXTL2) 464086(EXTL3)
PPS100977184(EXTL2) 100984535(EXTL3)
GGO101144361(EXTL3) 101153043(EXTL2)
PON100454000(EXTL2) 100458560(EXTL3)
NLE100585000(EXTL2) 100590557(EXTL3)
MCC100426593(EXTL3) 712189(EXTL2)
MCF101865433(EXTL3) 102129629(EXTL2)
CSAB103215549(EXTL3) 103224361(EXTL2)
CATY105579157(EXTL3) 105590136(EXTL2)
PANU101001089(EXTL3) 101013377(EXTL2)
TGE112630110(EXTL3) 112635537(EXTL2)
RRO104668244(EXTL3) 104673259(EXTL2)
RBB108524175(EXTL2) 108537365(EXTL3)
TFN117075444(EXTL2) 117094279(EXTL3)
PTEH111525765(EXTL2) 111545749(EXTL3)
CJC100400452(EXTL3) 100412156(EXTL2)
SBQ101037632(EXTL3) 101043244(EXTL2)
CSYR103252413(EXTL2) 103272211(EXTL3)
MMUR105862238(EXTL2) 105877144(EXTL3)
OGA100956398(EXTL3) 100959711(EXTL2)
MMU54616(Extl3) 58193(Extl2)
MCAL110291045(Extl2) 110309303(Extl3)
MPAH110320549(Extl2) 110325330(Extl3)
RNO310803(Extl2) 56819(Extl3)
MCOC116084271(Extl3) 116093673(Extl2)
MUN110551871(Extl2) 110559132(Extl3)
CGE100751999(Extl3) 100761218(Extl2)
PLEU114707292(Extl3) 114707427(Extl2)
NGI103729989(Extl3) 103741874(Extl2)
HGL101697847(Extl3) 101726394(Extl2)
CPOC100716129(Extl3) 100732343(Extl2)
CCAN109682613 109698077(Extl2)
DORD105991591(Extl3) 105997114(Extl2)
DSP122104161(Extl2) 122125248(Extl3)
NCAR124962564 124983066
OCU100348721(EXTL3) 100352420(EXTL2)
OPI101533966(EXTL2) 101536233(EXTL3)
TUP102472563(EXTL3) 102478415(EXTL2)
CFA479925(EXTL2) 486091(EXTL3)
VVP112918475(EXTL2) 112926771(EXTL3)
VLG121486912(EXTL2) 121496756(EXTL3)
AML100469588(EXTL3) 100480634(EXTL2)
UMR103669325(EXTL2) 103675952(EXTL3)
UAH113254344(EXTL2) 113269941(EXTL3)
UAR123777356(EXTL3) 123795152(EXTL2)
ELK111143177 111153998
LLV125092954 125098428
MPUF101674896(EXTL3) 101682970(EXTL2)
ORO101369733(EXTL2) 101377818(EXTL3)
EJU114208053(EXTL2) 114217463(EXTL3)
ZCA113918676(EXTL2) 113924330(EXTL3)
MLX118019078(EXTL2) 118022124(EXTL3)
FCA101089128(EXTL2) 101095491(EXTL3)
PYU121024917(EXTL3) 121027567(EXTL2)
PBG122474042(EXTL3) 122480841(EXTL2)
PTG102958499(EXTL2) 102961650(EXTL3)
PPAD109262363(EXTL3) 109271932(EXTL2)
AJU106976417(EXTL2) 106983017(EXTL3)
HHV120220154(EXTL3) 120226813(EXTL2)
BTA540527(EXTL2) 783970(EXTL3)
BOM102267911(EXTL2) 102273632(EXTL3)
BIU109556009(EXTL2) 109562800(EXTL3)
BBUB102398616(EXTL3) 102406549(EXTL2)
CHX102174617(EXTL2) 102178172(EXTL3)
OAS101108814(EXTL3) 101115079(EXTL2)
ODA120856787(EXTL2) 120860402(EXTL3)
CCAD122436631(EXTL2) 122452882(EXTL3)
SSC100152703(EXTL3) 100155305(EXTL2)
CFR102507432(EXTL3) 102518222(EXTL2)
CBAI105065361(EXTL2) 105079118(EXTL3)
CDK105084916(EXTL2) 105102072(EXTL3)
VPC102532676(EXTL3) 102537424(EXTL2)
BACU103001155(EXTL2) 103009480(EXTL3)
LVE103078702(EXTL3) 103089228(EXTL2)
OOR101285385(EXTL2) 101286194(EXTL3)
DLE111180111(EXTL2) 111185386(EXTL3)
PCAD102987427(EXTL2) 102995934(EXTL3)
PSIU116748773(EXTL2) 116755265(EXTL3)
ECB100050181(EXTL2) 100054778(EXTL3)
EPZ103550057(EXTL2) 103567372(EXTL3)
EAI106832529(EXTL3) 106837773(EXTL2)
MYB102249572(EXTL2) 102250952(EXTL3)
MYD102754802(EXTL2) 102764127(EXTL3)
MMYO118657784(EXTL3) 118673469(EXTL2)
MLF102437020(EXTL3) 102437553(EXTL2)
MNA107530682(EXTL2) 107537770(EXTL3)
PKL118710818(EXTL3) 118727287(EXTL2)
HAI109371361(EXTL2) 109379533(EXTL3)
DRO112312602(EXTL3) 112314076(EXTL2)
SHON118984343(EXTL3) 118990630(EXTL2)
AJM119053020(EXTL3) 119053021 119054948(EXTL2)
PDIC114488754(EXTL2) 114502307(EXTL3)
PHAS123804407(EXTL2) 123813737(EXTL3)
MMF118615157(EXTL3) 118637480(EXTL2)
RFQ117014462(EXTL2) 117037898(EXTL3)
PALE102883756(EXTL2) 102889624(EXTL3)
PGIG120607845(EXTL2) 120613246(EXTL3)
PVP105291510(EXTL3) 105297546(EXTL2)
RAY107504389(EXTL2) 107510781(EXTL3)
MJV108391555(EXTL3) 108407604(EXTL2)
TOD119235257(EXTL2) 119253967 119258027(EXTL3) 119259679
SARA101545694(EXTL2) 101556463(EXTL3)
LAV100658953(EXTL2) 100666182(EXTL3)
TMU101345745 101358076
DNM101431062(EXTL2) 101446669(EXTL3)
MDO100030828(EXTL3) 100032966(EXTL2)
GAS123234817(EXTL3) 123247092(EXTL2)
SHR100914736(EXTL2) 100919560(EXTL3)
PCW110209977(EXTL3) 110217549(EXTL2)
OAA100087229(EXTL3) 100091427(EXTL2)
GGA422022(EXTL3) 424465(EXTL2)
PCOC116235605(EXTL3) 116243553(EXTL2)
MGP100542317(EXTL3) 100549273(EXTL2)
CJO107312356(EXTL3) 107317515(EXTL2)
NMEL110396622(EXTL3) 110402398(EXTL2)
APLA101791181(EXTL3) 101792118(EXTL2)
ACYG106038151(EXTL3) 106039416(EXTL2)
AFUL116487389(EXTL3) 116491985(EXTL2)
TGU100228076(EXTL3) 100232258(EXTL2)
LSR110476418(EXTL3) 110477488(EXTL2)
SCAN103814860 103821407(EXTL3)
PMOA120496811(EXTL3) 120506668(EXTL2)
OTC121345221(EXTL2) 121347459(EXTL3)
PRUF121360869(EXTL3) 121362194(EXTL2)
GFR102041533(EXTL3)
FAB101818645(EXTL3)
PHI102105383(EXTL3) 102114416(EXTL2)
PMAJ107201996(EXTL3) 107208067(EXTL2)
CCAE111927356(EXTL3) 111932725(EXTL2)
CCW104696886(EXTL3) 109143175(EXTL2)
ETL114061647(EXTL2) 114068426(EXTL3)
ZAB102067303(EXTL2) 102071380(EXTL3)
FPG101917228(EXTL3) 101920946(EXTL2)
FCH102049911(EXTL2) 102056733(EXTL3)
CLV102085730(EXTL2) 102088676(EXTL3)
EGZ104127251(EXTL3)
NNI104014701(EXTL3) 104015519(EXTL2)
ACUN113478245(EXTL3) 113482992(EXTL2)
TALA104355903(EXTL2) 104357343(EXTL3)
PADL103913240(EXTL3)
ACHC115348769(EXTL2) 115350851(EXTL3)
AAM106488030(EXTL3) 106499747(EXTL2)
AROW112966640(EXTL3) 112966831(EXTL2)
NPD112946173(EXTL2) 112948868(EXTL3)
DNE112979807(EXTL2) 112984257(EXTL3)
ASN102377426(EXTL3) 102388723(EXTL2)
AMJ102573600(EXTL2) 102575775(EXTL3)
CPOO109307239(EXTL2) 109311530(EXTL3)
GGN109286887(EXTL2) 109300230(EXTL3)
PSS102462577(EXTL3) 102462903(EXTL2)
CMY102930795(EXTL2) 102945736(EXTL3)
CPIC101935250(EXTL3) 101945022(EXTL2)
TST117875421(EXTL3) 117881985(EXTL2)
CABI116831110(EXTL3) 116839218(EXTL2)
MRV120369843(EXTL2) 120401043(EXTL3)
ACS100557117(extl3) 100563405(extl2)
PVT110070203(EXTL3) 110076514(EXTL2)
SUND121920560(EXTL3) 121929446
PBI103050271(EXTL2) 103060383(EXTL3)
PMUR107284865(EXTL3) 107302497(EXTL2)
TSR106541899(EXTL3)
PGUT117667705(EXTL3) 117677267(EXTL2)
VKO123024597(EXTL2) 123033591(EXTL3)
PMUA114594927(EXTL3) 114598702(EXTL2)
ZVI118082960(EXTL3) 118089186(EXTL2)
GJA107106370(EXTL3) 107107448(EXTL2)
XLA443929(extl2.L) 444275(extl3.S) 447542(extl3.L)
XTR493327(extl2) 493398(extl3)
NPR108791470(EXTL2) 108802201(EXTL3)
RTEM120936038(EXTL3) 120945619(EXTL2)
BBUF120979351(EXTL2) 120997178(EXTL3)
BGAR122934492(EXTL3) 122943386(EXTL2)
DRE493783(extl3) 558373(extl2)
SRX107715497 107719384 107722455 107745900
SANH107669453 107681726(extl2) 107701317 107704067
SGH107548434 107559127
CCAR109106074(extl2) 109112811 109112812 109113082
CAUA113046026(extl2) 113068614 113120607
PPRM120471391(extl2) 120492082(extl3)
IPU108257719(extl3)
PHYP113532052(extl3)
SMEO124377167(extl3)
TFD113655598(extl3)
AMEX103035439(extl2) 103041905(extl3)
EEE113571699(extl3) 113584240(extl2)
TRU101066160(extl2) 101068405(extl3)
TNGGSTEN00023018G001 GSTEN00028414G001
LCO104934376(extl3) 104936574(extl2)
NCC104946504(extl3) 104951471(extl2)
CGOB115023052(extl2)
ELY117270963(extl3) 117272355(extl2)
PLEP121951875(extl2) 121955766(extl3)
SLUC116037280(extl2) 116065957(extl3)
ECRA117954238(extl2) 117961987(extl3)
PFLV114564817(extl2) 114573362(extl3)
GAT120807950(extl3) 120815639(extl2)
PPUG119194732(extl3) 119203640(extl2)
MSAM119902150(extl2) 119908552(extl3)
CUD121520559(extl2) 121521448(extl3)
MZE101467370(extl3) 101469432(extl2)
ONL100694061(extl3) 100711437(extl2)
OAU116312735(extl3) 116332828(extl2)
OLA101163453(extl3) 101174221(extl2)
OML112139608(extl2) 112157675(extl3)
XMA102236630(extl2) 102237533(extl3)
XCO114146128(extl2) 114158236(extl3)
XHE116721283(extl2) 116734009(extl3)
PRET103457379(extl3) 103479408(extl2)
PFOR103133526(extl3) 103148289(extl2)
PLAI106948958(extl2) 106962939(extl3)
PMEI106911334(extl3) 106925675(extl2)
GAF122825437(extl3) 122840814(extl2)
CVG107081098(extl3) 107083757(extl2)
CTUL119771555(extl3) 119786166(extl2)
GMU124870006(extl2) 124882424(extl3)
NFU107377723(extl3) 107384011(extl2)
KMR108233500(extl3) 108239591(extl2)
ALIM106525671(extl3) 106535008(extl2)
NWH119415130(extl2) 119418590(extl3)
AOCE111572767(extl2) 111583150(extl3)
CSEM103381243(extl3) 103396080(extl2)
POV109636091(extl3) 109640876(extl2)
SSEN122780197(extl2) 122783726(extl3)
HHIP117758102(extl3) 117777956(extl2)
LCF108873476(extl3) 108883513(extl2)
SDU111229104(extl2) 111236334(extl3)
SLAL111654468(extl2) 111661394(extl3)
XGL120789157(extl3) 120799059(extl2)
HCQ109514670(extl3)
BPEC110158086(extl2) 110164237(extl3)
MALB109957690(extl2) 109962352(extl3)
SASA100380460(extl2) 106571659 106608199
OTW112218009 112227209(extl2) 112250478
OMY110509057(extl2) 110522550 110529776
OGO123990909 123995375 124042121(extl2)
ONE115103346(extl2) 115107913 115139163
SALP111954206 111978960(extl2) 112069087
SNH120019129 120020734(extl2) 120024042
ELS105007545(extl2) 105017562(extl3)
SFM108924136(extl3) 108935058(extl2)
PKI111841187(extl3)
AANG118228902(extl2) 118229494(extl3)
LOC102682682(extl2) 102686314(extl3)
PSPA121316024(extl3) 121330853(extl2)
ARUT117403267(extl3) 117405024(extl2)
LCM102348020(EXTL2) 102361988(EXTL3)
CMK103188516 103188850(extl2) 121848587
RTP109926989(extl3) 109936576(extl2)
BFO118404540
BBEL109483146
CIN100184170
SCLV120330866
SPU585810
APLC110979510 110979551 110979555
SKO100369934
DMEDmel_CG15110(botv)
DER6547292
DSE6609894
DSIDsimw501_GD11429(Dsim_GD11429)
DYADyak_GE12019
DAN6494354
DSR110184038
DPODpse_GA13499 Dpse_GA25148
DPE6601544 6601546
DMN108160133 117187784
DWI6646203
DGR6568946
DMODmoj_GI18912
DAZ108616969
DNV108653682
DHE111594305
DVI6635366
CCAT101461942
BOD106617525
MDE101892286
SCAC106080711
LCQ111688369
AGAAgaP_AGAP001688
ACOZ120949727
AARA120893798
AAG5578446
AALB109398955
CQUCpipJ_CPIJ016867
CPII120413984
CNS116339565
ACER108001564
ALAB122711813
BIM105680701
BBIF117205178
BVK117243560
BVAN117164164
BTER105666123
BPYO122571851
CCAL108622898
OBB114871977
MGEN117219922
CGIG122400966
SOC105200907
MPHA105838726
AEC105154628
ACEP105620069
PBAR105433557
VEM105564962
HST105188784
DQU106745718
CFO105251656
FEX115234785
LHU105674049
PGC109853827
OBO105288161
PCF106786799
PFUC122522139
VPS122638200
NVI100121031
CSOL105365204
TPRE106651356
MDL103569026
CGLO123261632
FAS105262784
DAM107043534
AGIF122847424
CCIN107268133
TCA663383
DPA109537502
SOY115881569
ATD109596360
CSET123311160
AGB108912068
LDC111503945
NVL108567151
APLN108735767
PPYR116171124
OTU111427848
BMOR101742984
BMAN114248987
MSEX115450427
DPLKGM_208641
BANY112045658
PMAC106712471
PPOT106107976
PXU106117396
PRAP111000541
ZCE119830202
HAW110370155
TNL113493550
SLIU111355951
OFU114352184
PXY105388581
API100167784
DNX107163206
AGS114122118
RMD113556372
BTAB109030556
DCI103516295
CLEC106672266
HHAL106679847
NLU111049614
PHUPhum_PHUM490250
FOC113203130
ZNE110831980
CSEC111866806
FCD110846056
DPXDAPPUDRAFT_218850
DMK116933567
PVM113819647
PJA122245774
PCHN125027164
HAME121874923
PCLA123762596
PTRU123501943
HAZT108665016
EAF111718430
ISCIscW_ISCW011872
DSV119461512 119461515
RSAN119396015
RMP119174327
VDE111246358
VJA111268050
TUT107366477
DPTE113795874
DFR124491918
CSCU111639700
PTEP107455291
SDM118192709
CELCELE_K01G5.6(rib-2)
CBRCBG_18318(Cbr-rib-2)
BMYBM_BM4732(Bm4732)
LOALOAG_07208
NAINECAME_03535
TSPTsp_02636
HROHELRODRAFT_83262
LGILOTGIDRAFT_137450
PCAN112562383
BGT106070127
GAE121377129
HRF124114597
HRJ124256095
CRG105341576
MYI110442614
PMAX117316492
MMER123540528 123543849
OBI106873155 106874784
OSN115223438 115223625 115223714 115231411
LAK106152057
SMMSmp_073220
OVIT265_06473 T265_07036
EGLEGR_06675 EGR_08543
NVE5504655
EPA110234330
ATEN116293188
ADF107341811
AMIL114970312
PDAM113677366
SPIS111325684
DGT114527489
XEN124456299
HMG100212160
AQU100633921 100636716 100640795 105313463 105313464 105314839 105316428 109580906 109580910
CCPCHC_T00009229001
FGRFGSG_12261
TRETRIREDRAFT_56638
TRRM419DRAFT_108298
CMTCCM_02315
VALVDBG_08449
ZTRMYCGRDRAFT_107566
MBRMONBRDRAFT_25145
SREPTSG_09573
EHXEMIHUDRAFT_54630 EMIHUDRAFT_74009
GTTGUITHDRAFT_143314
TCR510279.240
TVATVAG_110260
 » show all
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
LinkDB

DBGET integrated database retrieval system