KEGG   ENZYME: 2.4.1.224
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
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)
R07334 > R05936(G) R10139(G)
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, glucuronosyl-galactosyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase.
History
EC 2.4.1.224 created 2002
Pathway
ec00534  Glycosaminoglycan biosynthesis - heparan sulfate / heparin
ec01100  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
HSA2131(EXT1) 2132(EXT2) 2134(EXTL1) 2137(EXTL3)
PTR107976420(EXT1) 451140(EXT2) 464086(EXTL3) 469238(EXTL1)
PPS100975712(EXT2) 100979888(EXTL1) 100984535(EXTL3) 100985695(EXT1)
GGO101130563(EXT2) 101144361(EXTL3) 101149310(EXTL1) 109025298(EXT1)
PON100172231(EXTL1) 100172450(EXT1) 100436160(EXT2) 100458560(EXTL3)
NLE100590557(EXTL3) 100597702(EXT1) 100604250(EXT2) 105738035(EXTL1)
MCC100426593(EXTL3) 701488(EXT1) 713664(EXTL1) 715975(EXT2)
MCF101865433(EXTL3) 101926231(EXT1) 102125916(EXTL1) 102136104(EXT2)
CSAB103215549(EXTL3) 103225338(EXTL1) 103236980 103237338(EXT1)
CATY105579157(EXTL3) 105584539(EXT1) 105586840(EXT2) 105594948(EXTL1)
PANU100137439(EXT1) 101001089(EXTL3) 101009465(EXTL1) 101019229(EXT2) 116272456 116272614
TGE112606187(EXT2) 112608368(EXTL1) 112629920(EXT1) 112630110(EXTL3)
RRO104656082(EXTL1) 104658897(EXT1) 104668244(EXTL3) 104673882(EXT2)
RBB108518038(EXTL1) 108519255(EXT2) 108536176(EXT1) 108537365(EXTL3)
TFN117079472(EXT1) 117081393(EXT2) 117093079(EXTL1) 117094279(EXTL3)
PTEH111544934(EXT2) 111545749(EXTL3) 111547509(EXTL1) 111551020(EXT1)
CJC100386964(EXT1) 100400452(EXTL3) 100400679(EXT2) 100412852(EXTL1)
SBQ101037632(EXTL3) 101039408(EXT2) 101053138(EXTL1) 101053444(EXT1)
CSYR103260803 103263660 103264178 103267603(EXTL1) 103272211(EXTL3) 103272774
MMUR105863568(EXTL1) 105863703(EXT1) 105877144(EXTL3) 105885490(EXT2)
OGA100940987(EXTL1) 100943601(EXT1) 100949922(EXT2) 100956398(EXTL3)
MMU14042(Ext1) 14043(Ext2) 54616(Extl3) 56219(Extl1)
MCAL110292428(Extl1) 110309303(Extl3) 110310432(Ext1) 110311448(Ext2)
MPAH110317782(Ext2) 110323450(Extl1) 110325330(Extl3) 110334870(Ext1)
RNO299907(Ext1) 311215(Ext2) 313610(Extl1) 56819(Extl3)
MCOC116082489(Ext1) 116084271(Extl3) 116090518(Ext2) 116096808(Extl1)
MUN110540376(Ext2) 110552198(Extl1) 110559132(Extl3) 110559447 110559448
CGE100689334(Ext1) 100751585(Ext2) 100751999(Extl3) 100770000(Extl1)
PLEU114694845(Ext1) 114697258 114699729(Ext2) 114705983 114707292(Extl3)
NGI103729989(Extl3) 103737100(Extl1) 103738135(Ext1) 103751554(Ext2)
HGL101697847(Extl3) 101705292(Extl1) 101706257(Ext1) 101715817(Ext2)
CPOC100712625(Ext2) 100716129(Extl3) 100716132(Ext1) 100732210(Extl1)
CCAN109682613 109683077(Extl1) 109686418 109702003
DORD105991591(Extl3) 105996073(Ext2) 105997725(Ext1) 105998840(Extl1)
DSP122100734(Ext2) 122102360(Ext1) 122115278(Extl1) 122125248(Extl3)
NCAR124960112 124961920 124981119 124983066
OCU100343697(EXT2) 100347418(EXTL1) 100348721(EXTL3) 100351235(EXT1)
OPI101533034(EXTL1) 101535829(EXT1) 101536088(EXT2) 101536233(EXTL3)
TUP102467803(EXT1) 102472563(EXTL3) 102487057(EXT2) 102499532(EXTL1)
CFA475989(EXT2) 482024(EXT1) 486091(EXTL3) 487363(EXTL1)
VVP112915941(EXT1) 112924118(EXT2) 112926771(EXTL3) 112931669(EXTL1)
VLG121496756(EXTL3) 121497437(EXTL1) 121498758(EXT1) 121501521(EXT2)
AML100465258(EXT1) 100469588(EXTL3) 100482562(EXTL1) 105242037(EXT2)
UMR103661582(EXT1) 103666523(EXTL1) 103672344(EXT2) 103675952(EXTL3)
UAH113252791(EXT1) 113265011(EXT2) 113268473(EXTL1) 113269941(EXTL3)
UAR123777356(EXTL3) 123782477(EXTL1) 123797768(EXT1) 123799595(EXT2)
ELK111146555 111150031 111153998 111154894
LLV125078631 125092954 125097162 125098595
MPUF101672930(EXT2) 101674896(EXTL3) 101683113(EXTL1) 101691546(EXT1)
ORO101366478(EXT2) 101377818(EXTL3) 101379569(EXTL1) 101382133(EXT1)
EJU114199089(EXT1) 114215287(EXT2) 114217203(EXTL1) 114217463(EXTL3)
ZCA113913370(EXTL1) 113914556(EXT2) 113924330(EXTL3) 113931105(EXT1)
MLX118015150(EXT1) 118015783(EXT2) 118019850(EXTL1) 118022124(EXTL3)
FCA101081362(EXT1) 101083620(EXT2) 101083693(EXTL1) 101095491(EXTL3)
PYU121023608(EXTL1) 121024917(EXTL3) 121042894(EXT1) 121043515(EXT2)
PBG122474042(EXTL3) 122480109(EXTL1) 122483728(EXT2) 122495739(EXT1)
PTG102961650(EXTL3) 102962317(EXTL1) 102964333(EXT2) 102968916(EXT1)
PPAD109256806(EXTL1) 109262363(EXTL3) 109273237(EXT1) 109279210 109279211(EXT2)
AJU106975536(EXT1) 106977618(EXTL1) 106978049(EXT2) 106983017(EXTL3)
HHV120220154(EXTL3) 120228461(EXT2) 120228668(EXT1) 120230901(EXTL1)
BTA281151(EXT2) 511060(EXTL1) 538602(EXT1) 783970(EXTL3)
BOM102267135(EXTL1) 102273632(EXTL3) 102275740(EXT2) 102280308(EXT1)
BIU109562800(EXTL3) 109568471(EXT1) 109569634(EXT2) 109573580(EXTL1)
BBUB102389161(EXT2) 102398616(EXTL3) 102407802(EXT1) 102415224(EXTL1)
CHX102178172(EXTL3) 102182004(EXTL1) 102182243(EXT2) 102182809(EXT1)
OAS101108814(EXTL3) 101110867(EXT2) 101113394(EXTL1) 101114877(EXT1)
ODA120855039(EXTL1) 120860402(EXTL3) 120860718 120876908(EXT2) 120878901(EXT1)
CCAD122426536(EXTL1) 122449196(EXT2) 122450981(EXT1) 122452882(EXTL3)
SSC100152703(EXTL3) 100157655(EXT1) 100522362(EXT2) 100623848(EXTL1)
CFR102507432(EXTL3) 102508456(EXT1) 102514905(EXT2) 102521830(EXTL1)
CBAI105063480(EXTL1) 105076487(EXT2) 105079118(EXTL3) 105082972(EXT1)
CDK105096148(EXT1) 105100672(EXTL1) 105102072(EXTL3) 105106838(EXT2)
VPC102532676(EXTL3) 102535018(EXT1) 102542570(EXT2) 102544441(EXTL1)
BACU102999728(EXT2) 103004222(EXTL1) 103009480(EXTL3) 103019432(EXT1)
LVE103068452(EXTL1) 103078702(EXTL3) 103078738(EXT1) 103091009(EXT2)
OOR101280958(EXT2) 101281062(EXTL1) 101286194(EXTL3) 101286251(EXT1)
DLE111163234(EXTL1) 111165183(EXT1) 111184011(EXT2) 111185386(EXTL3)
PCAD102979294 102980097(EXT1) 102983199(EXTL1) 102995934(EXTL3)
PSIU116738709(EXTL1) 116742524(EXT1) 116755265(EXTL3) 116758175(EXT2)
ECB100054778(EXTL3) 100055879(EXT2) 100056983(EXT1) 100071242(EXTL1)
EPZ103549478(EXT1) 103556459(EXT2) 103556774(EXTL1) 103567372(EXTL3)
EAI106832529(EXTL3) 106839736(EXT1) 106839934(EXTL1) 106843971(EXT2)
MYB102238781(EXT2) 102244714(EXT1) 102246778(EXTL1) 102250952(EXTL3)
MYD102761567(EXTL1) 102764127(EXTL3) 102765321(EXT1) 102767712(EXT2)
MMYO118657784(EXTL3) 118665109(EXT2) 118670761(EXT1) 118676894(EXTL1)
MLF102422014(EXT2) 102429540(EXTL1) 102437020(EXTL3) 102441376(EXT1)
MNA107525491(EXT2) 107533185(EXT1) 107537770(EXTL3) 107541891(EXTL1)
PKL118710818(EXTL3) 118712503 118718227(EXT1) 118723646(EXTL1)
HAI109379533(EXTL3) 109382533 109386781(EXTL1) 109391972(EXT2)
DRO112299237(EXTL1) 112303473(EXT2) 112312602(EXTL3) 112315333(EXT1)
SHON118977808(EXTL1) 118984343(EXTL3) 118988619(EXT1) 119002984(EXT2)
AJM119042253(EXTL1) 119051826(EXT1) 119053020(EXTL3) 119053021 119064418(EXT2)
PDIC114498143(EXTL1) 114499874(EXT2) 114502307(EXTL3) 114514407(EXT1)
PHAS123810129(EXT2) 123813737(EXTL3) 123832033(EXTL1) 123833269(EXT1)
MMF118615157(EXTL3) 118627094(EXTL1) 118627865(EXT2) 118631561(EXT1)
RFQ117027039(EXTL1) 117029882(EXT2) 117033795(EXT1) 117037898(EXTL3)
PALE102889624(EXTL3) 102890634(EXT2) 102891150(EXT1) 102894796(EXTL1)
PGIG120595391(EXT1) 120596043(EXTL1) 120606317 120613246(EXTL3)
PVP105290068(EXT2) 105290678(EXTL1) 105291510(EXTL3) 105292239(EXT1)
RAY107502529(EXTL1) 107507149(EXT1) 107510781(EXTL3) 107511887(EXT2)
MJV108386769 108391555(EXTL3) 108394755(EXT2) 108398779(EXTL1) 108399086(EXT1) 108399399
TOD119236338(EXTL1) 119238592 119255341(EXT2) 119258027(EXTL3)
SARA101538625(EXT1) 101551336(EXT2) 101555783(EXTL1) 101556463(EXTL3)
LAV100656016(EXT2) 100657623(EXT1) 100666182(EXTL3) 100673302(EXTL1)
TMU101348092 101358076 101361975 101362012
DNM101426325(EXTL1) 101436351(EXT1) 101436915(EXT2) 101446669(EXTL3)
MDO100010947(EXT2) 100015039(EXTL1) 100025396(EXT1) 100030828(EXTL3)
GAS123234817(EXTL3) 123241026(EXTL1) 123241182(EXT1) 123252570 123252571
SHR100918977(EXTL1) 100919560(EXTL3) 100929895(EXT1) 105750949(EXT2)
PCW110200303(EXT1) 110206108(EXTL1) 110209977(EXTL3) 110223945(EXT2)
OAA100078206(EXT1) 100079286(EXT2) 100087229(EXTL3)
GGA100858205(EXT1) 100859367(EXTL1) 422022(EXTL3) 425859(EXT2)
PCOC116227694(EXT1) 116229130(EXT2) 116230047(EXTL1) 116235605(EXTL3)
MGP100542119(EXT1) 100542317(EXTL3) 100545109 100551486(EXTL1)
CJO107310492(EXT1) 107312356(EXTL3) 107314816(EXT2) 107324063(EXTL1)
NMEL110387476(EXTL1) 110395119(EXT1) 110396622(EXTL3) 110401311(EXT2)
APLA101790687(EXT2) 101791181(EXTL3) 101792373(EXT1)
ACYG106037911(EXTL1) 106038151(EXTL3) 106038456(EXT1) 106044125(EXT2)
AFUL116485915(EXT1) 116487389(EXTL3) 116489660(EXT2) 116498028(EXTL1)
TGU100227900(EXT1) 100228076(EXTL3) 100231605(EXT2) 115498288(EXTL1)
LSR110472037(EXT2) 110472893(EXTL1) 110476418(EXTL3) 110479282(EXT1)
SCAN103814231(EXT1) 103821407(EXTL3) 103823579(EXTL1) 103826716(EXT2)
PMOA120496811(EXTL3) 120505159(EXT1) 120512558(EXT2) 120513889(EXTL1)
OTC121333361(EXT2) 121337462(EXT1) 121342741(EXTL1) 121347459(EXTL3)
PRUF121349516(EXT2) 121351215(EXTL1) 121355356(EXT1) 121360869(EXTL3)
GFR102035022(EXTL1) 102040196(EXT2) 102041533(EXTL3) 102043948(EXT1)
FAB101814666(EXT2) 101816822(EXT1) 101818645(EXTL3) 101818678(EXTL1)
PHI102099981(EXT1) 102105383(EXTL3) 102107359(EXT2) 102111356(EXTL1)
PMAJ107200137(EXT1) 107201996(EXTL3) 107205846(EXT2) 107213996(EXTL1)
CCAE111925064(EXT1) 111927356(EXTL3) 111930141(EXT2)
CCW104692676(EXT1) 104695274(EXT2) 104696886(EXTL3)
ETL114057979(EXT1) 114064860(EXT2) 114068426(EXTL3) 114070342(EXTL1)
ZAB102062154(EXT2) 102067827(EXT1) 102071380(EXTL3)
FPG101917228(EXTL3) 101919384(EXT1) 101922176(EXTL1) 101923547(EXT2)
FCH102049940(EXT1) 102054426(EXT2) 102056733(EXTL3) 102056866(EXTL1)
CLV102084082(EXTL1) 102085197(EXT2) 102088676(EXTL3) 102091811(EXT1)
EGZ104123693(EXT1) 104127251(EXTL3) 104134053(EXT2) 104134955(EXTL1)
NNI104014701(EXTL3) 104017847(EXTL1) 104018021(EXT1) 104019563(EXT2)
ACUN113476897(EXT1) 113478245(EXTL3) 113480641(EXT2) 113488189(EXTL1)
TALA104357343(EXTL3) 104365235(EXT1) 104367927(EXT2) 116964263(EXTL1)
PADL103913240(EXTL3) 103914958(EXT1) 103921685(EXT2)
ACHC115333408(EXT2) 115340329(EXT1) 115350851(EXTL3) 115351760(EXTL1)
AAM106485009 106488030(EXTL3) 106496271(EXT1) 106498294(EXT2) 106499622
AROW112960952(EXT1) 112964418(EXTL1) 112966640(EXTL3) 112972955 112978433(EXT2)
NPD112943491(EXT2) 112948502(EXT1) 112948868(EXTL3) 112950897(EXTL1)
DNE112979229(EXT2) 112982032(EXTL1) 112984257(EXTL3) 112989149(EXT1)
ASN102370729(EXT1) 102376854(EXTL1) 102377426(EXTL3) 102379048(EXT2)
AMJ102560415(EXT1) 102562202(EXT2) 102575775(EXTL3) 102575942(EXTL1)
CPOO109311530(EXTL3) 109318544(EXTL1) 109321140(EXT2) 109321481(EXT1)
GGN109294162(EXTL1) 109298936(EXT2) 109299004(EXT1) 109300230(EXTL3)
PSS102444477(EXT1) 102446763(EXT2) 102462577(EXTL3)
CMY102929859(EXTL1) 102938478(EXT2) 102939595(EXT1) 102945736(EXTL3)
CPIC101935250(EXTL3) 101946173(EXT1) 101949276(EXT2) 101953823(EXTL1) 103307378
TST117868243(EXTL1) 117873767(EXT1) 117875421(EXTL3) 117876973(EXT2)
CABI116818811(EXT1) 116830766(EXT2) 116831110(EXTL3) 116837997(EXTL1)
MRV120389310(EXTL1) 120398808(EXT1) 120401043(EXTL3) 120404543(EXT2)
ACS100553800(extl1) 100555771(ext2) 100557117(extl3) 100567014(ext1)
PVT110070203(EXTL3) 110076972(EXT1) 110081549(EXT2) 110084159(EXTL1)
SUND121915625(EXTL1) 121920560(EXTL3) 121928875(EXT1) 121933481(EXT2)
PBI103048904 103054708 103060383(EXTL3) 103062807(EXT2) 103067611(EXTL1)
PMUR107284865(EXTL3) 107292260 107295666(EXTL1) 107302595(EXT2)
TSR106538947 106539688(EXTL1) 106541899(EXTL3) 106545012(EXT2)
PGUT117663388(EXTL1) 117665492(EXT2) 117667705(EXTL3) 117668371(EXT1)
VKO123019173(EXT1) 123025901(EXTL1) 123026241(EXT2) 123033591(EXTL3)
PMUA114594927(EXTL3) 114596253(EXT2) 114601312(EXT1) 114601546(EXTL1)
ZVI118082960(EXTL3) 118085381(EXT2) 118086449(EXTL1) 118089708(EXT1)
GJA107106370(EXTL3) 107110827(EXTL1) 107113379 107122615(EXT2)
XLA380140(ext2.L) 398214(ext1.L) 398750(ext2.S) 399115(ext1.S) 444275(extl3.S) 447542(extl3.L)
XTR100151709(ext2) 448393(ext1) 493398(extl3)
NPR108786158(EXT2) 108790563(EXT1) 108802201(EXTL3)
RTEM120917049(EXT2) 120936038(EXTL3) 120941479(EXT1)
BBUF120980295(EXT2) 120997178(EXTL3) 121001066(EXT1)
BGAR122920720(EXT2) 122934492(EXTL3) 122938154(EXT1)
DRE101885478 493780(ext2) 493783(extl3) 497279(ext1a) 497280(ext1b) 497281(ext1c)
SRX107706235(extl1) 107706241 107711539 107715577 107719384 107727350 107733302 107745900 107750554 107754264 107754269 107754691
SANH107654722 107654729 107659874 107669453 107671371 107671458 107683605 107685778 107688159(ext2) 107688215 107688378 107696533 107704067
SGH107548434 107558762 107559127 107562300 107586005 107592048(extl1) 107592050 107592164 107592487 107592940 107592945 107593432
CCAR109050531 109056569 109059721 109072430 109074535(ext2) 109075419 109075422 109093562 109106263 109109198 109109965 109111718 109112811 109112812 109113082
CAUA113052033 113062235 113062294 113062301 113063437 113068614 113073234 113083119 113105758 113115689 113119245 113119254 113120607 113121224
PPRM120468007(ext2) 120469966(ext1c) 120479262(ext1b) 120491188(ext1a) 120492082(extl3) 120493730
IPU108256617(ext1a) 108257122(ext1b) 108257140 108257719(extl3) 108271344(ext2) 108275270
PHYP113529295 113532052(extl3) 113532714 113535947 113535988 113536365(ext2)
SMEO124375785(ext1b) 124375938 124377167(extl3) 124390802(ext2) 124401370 124403868(ext1a)
TFD113643961(ext1c) 113649722(ext1a) 113654795 113655598(extl3) 113661608(ext1b) 113663258(ext2)
AMEX103026919 103031914 103040053 103041905(extl3) 103046596(ext2) 103047171 111197650
EEE113571699(extl3) 113575759(ext2) 113578814 113588018(ext1) 113591568 113591687
TRU101065654 101066048(ext2) 101068405(extl3) 101072426 101072822 101075972
TNGGSTEN00017827G001 GSTEN00023018G001 GSTEN00032917G001 GSTEN00032935G001 GSTEN00035381G001 GSTEN00038113G001
LCO104919199(ext2) 104926220 104931682 104932462 104934376(extl3) 104937142
NCC104945394 104946504(extl3) 104949435 104961795(ext1) 104963025(ext2)
CGOB115003439 115006327(ext2) 115015568 115016180 115021154
ELY117258719(ext2) 117263289(ext1b) 117263650 117265950 117270842 117270963(extl3)
PLEP121945585 121955317 121955766(extl3) 121958010(ext1b) 121958038 121960250(ext2)
SLUC116034014(ext2) 116035281 116047237(ext1c) 116047249(ext1b) 116065957(extl3) 116066120
ECRA117943850(ext2) 117946948 117956425(ext1b) 117956791 117961813 117961814 117961987(extl3)
PFLV114553112(ext2) 114557177 114567744 114567814 114572953 114573362(extl3)
GAT120807940 120807950(extl3) 120808600(ext2) 120810156 120826358(ext1b) 120826673(ext1c)
PPUG119194732(extl3) 119195167 119197352 119219177(ext1b) 119219200 119229335(ext2)
MSAM119890106(ext2) 119897919 119907162 119907458(ext1b) 119908552(extl3) 119908630 119909485
CUD121513315 121513674(ext2) 121521338 121521448(extl3) 121523384(ext1b) 121524170
MZE101464589 101467370(extl3) 101473582(ext2) 101479111 101482519 101486492
ONL100694061(extl3) 100695641(ext2) 100700775 100705583 100707723 109194193
OAU116309414 116312735(extl3) 116318909 116318948(ext1b) 116322583 116327190(ext2)
OLA101163398(ext2) 101163453(extl3) 101164181 101167217 101169374
OML112143494 112157675(extl3) 112160904(ext2) 112162700(ext1b) 112162739(ext1c)
XMA102217307 102217354 102230266 102234444(ext2) 102237506 102237533(extl3)
XCO114141516 114142777(ext2) 114155469 114156156 114158236(extl3) 114159116
XHE116716748 116718349(ext2) 116731149 116731796 116734009(extl3) 116734657
PRET103457265 103457379(extl3) 103462432(ext2) 103472886(ext1) 103473086 103478346
PFOR103131421 103133526(extl3) 103135121 103139431(ext2) 103151922 103154987 103155379(ext1)
PLAI106951280 106953583(ext1) 106958190(ext2) 106961006 106962939(extl3) 106964918
PMEI106907411 106911334(extl3) 106923397(ext1) 106926106(ext2) 106928852
GAF122821865(ext2) 122825094 122825437(extl3) 122831600 122843906(ext1b) 122844227(ext1c)
CVG107081098(extl3) 107082854 107085591(ext2) 107086154 107089629(ext1) 107094248 107094935
CTUL119771555(extl3) 119772289(ext1c) 119775297 119777081 119778046(ext1b) 119782724(ext2)
GMU124865747 124866743(ext2) 124879655(ext1c) 124879738(ext1b) 124881887 124882424(extl3)
NFU107377723(extl3) 107377760 107378775 107381443(ext2) 107394502 107394966
KMR108228674(ext2) 108232308 108233500(extl3) 108243943(ext1b) 108244864 108250176(ext1c)
ALIM106511501 106514225(ext1) 106525671(extl3) 106527530 106530767(ext2) 106532427 106535689
NWH119418590(extl3) 119418618 119425471 119425560(ext1c) 119427822(ext2)
AOCE111563665 111564587 111574611(ext2) 111580481 111583150(extl3) 111589114(ext1)
CSEM103378751(ext2) 103381137 103381243(extl3) 103388612(ext1) 103393992 103394040
POV109632177(ext1) 109636091(extl3) 109636768(ext2) 109639984 109642456 109643173 109644500
SSEN122772353(ext2) 122774807 122783726(extl3) 122784514 122786453 122786792
HHIP117758031 117758102(extl3) 117758296(ext2) 117770077 117770079(ext1b) 117776501
LCF108873476(extl3) 108888686 108889564 108889565 108889668(ext2) 108889677 108891202
SDU111222995 111223271 111227735 111236334(extl3) 111237872 111238419(ext2)
SLAL111654812(ext2) 111658101 111661394(extl3) 111671857 111671874 111672565
XGL120784183 120786451(ext1b) 120786592(ext1c) 120788958 120789157(extl3) 120794269(ext2)
HCQ109514394(ext2) 109514670(extl3) 109518055 109518060 109523479 109529689(ext1)
BPEC110153759 110156338 110164237(extl3) 110171534 110172856(ext2) 110173279
MALB109952487 109958190 109958643 109958685 109962352(extl3) 109965798(ext2) 109968834
SASA100306779(ext1c) 100380322(ext2) 106570050 106571404 106571659 106588496 106588504 106594121 106597672 106607809 106608199
OTW112217339 112218009 112229926 112229932 112247034 112250171 112250478 112264222 112264884 112264892 121844920
OMY110487634 110488385 110496112(ext1b) 110496808(ext1c) 110506669 110511734 110522229 110522550 110526537 110529611 110529776
OGO123990750 123990909 123995089 123995375 124012587 124012858(ext1b) 124017649 124033638 124035622 124043635 124043639
ONE115107751 115107913 115112435(ext2) 115128292 115128299 115131708 115131715 115139163 115139415
SALP111954206 111954432 111955132 111955294 111955814 111955850 111969484 111972462 112069087
SNH120019129 120019504 120022446 120022523 120024042 120024563 120027266 120027289 120040883 120049131
ELS105005807 105017562(extl3) 105017786 105022258(ext2) 105023503 105027867
SFM108922050(ext1) 108924136(extl3) 108928562 108931808 108934682(ext2) 108940524
PKI111836091 111840708(ext2) 111841187(extl3) 111843930 111843931 111844061 111854291
AANG118215430(ext2) 118226079 118229494(extl3) 118230580 118233246(ext1c) 118233844
LOC102686314(extl3) 102688650 102688999(ext1) 102689027(ext2) 102698389
PSPA121294359 121303296 121308213 121313281 121314898 121315561 121316024(extl3) 121317487 121329636
ARUT117394872 117400267 117402503 117403267(extl3) 117411447 117412720 117414200 117432282 117435140 117964062
LCM102353460(EXT1) 102354369 102354854(EXTL1) 102361988(EXTL3) 102363354(EXT2)
CMK103174976(ext2) 103179627 103184956 103185802 103188516 121848587
RTP109910890(ext2) 109926989(extl3) 109930524 109932300
BFO118404100 118404540 118408274
BBEL109479557 109480278 109483146
CIN100176235 100184170 100185120
SCLV120330866 120335452 120335699
SPU100893938 115918716 577995 585810
APLC110979510 110979551 110979555 110981069 110986223
SKO100313593 100369934 100372539 102809744
DMEDmel_CG10117(ttv) Dmel_CG15110(botv) Dmel_CG8433(sotv)
DER6547292 6548926 6549168
DSE6609157 6609382 6609894
DSIDsimw501_GD11060(Dsim_GD11060) Dsimw501_GD11429(Dsim_GD11429) Dsimw501_GD25567(Dsim_GD25567)
DYADyak_GE12019 Dyak_GE13596 Dyak_GE14097
DAN6494354 6495892 6496091
DSR110177800 110178267 110184038
DPODpse_GA10084 Dpse_GA13499 Dpse_GA17244 Dpse_GA25148
DPE6590702 6590872 6601544 6601546
DMN108159562 108159662 108160133 117187784 117190510
DWI6640721 6640835 6646203
DGR6560884 6561214 6568946
DMODmoj_GI18912 Dmoj_GI20737 Dmoj_GI20872
DAZ108615183 108615817 108616969
DNV108652943 108653682 115564114 115564922
DHE111594305 111600127 111602550
DVI6624746 6627192 6635366
CCAT101453656 101460876 101461942
BOD106617525 106617537 106618671
MDE101888783 101892286 101894872 101901385
SCAC106080711 106082419 106091369
LCQ111677016 111677307 111688369
AGAAgaP_AGAP000081 AgaP_AGAP001688 AgaP_AGAP010388
ACOZ120949727 120952216 120956752
AARA120893798 120901724 120906198
AAG5566768 5578353 5578446
AALB109397881 109398955 109411435 109416900 109428863 115254192
CQUCpipJ_CPIJ009765 CpipJ_CPIJ012734 CpipJ_CPIJ016867
CPII120413984 120424304 120429091
CNS116339565 116340037 116349472
AME408293 726216
ACER107997927 107999513 108001564
ALAB122711813 122711993 122714818
BIM100741317 100744382 105680701
BBIF117205178 117211962 117214192
BVK117232255 117239137 117243560
BVAN117156137 117164164 117166073
BTER100643223 100650849 105666123
BPYO122571366 122571851 122577021
CCAL108622677 108622898 108627221
OBB114871565 114871663 114871977 114880970
MGEN117219922 117220187 117224160
NMEA116429461 116432549
CGIG122399622 122400966 122404430
SOC105195910 105198634 105200907
MPHA105835292 105838726 105839250
AEC105150811 105153382 105154628
ACEP105619491 105620069 105627211
PBAR105425236 105427034 105433557
VEM105564271 105564746 105564962
HST105181013 105188235 105188784
DQU106742285 106745718 106750617
CFO105251656 105253055 105253243
FEX115234785 115235169 115240513
LHU105673709 105674049 105675813
PGC109853827 109854839 109863645
OBO105287638 105288161
PCF106786799 106790860 106791047
PFUC122518599 122522139 122523426
VPS122629883 122631850 122638200
NVI100114642 100120177 100121031
CSOL105364815 105365204 105366129
TPRE106651356 106653398
MDL103569026 103578696 103580382
CGLO123261632 123268665 123275072
FAS105262784 105265574 105268060
DAM107040372 107041901 107043534
AGIF122847424 122851929 122854354
CCIN107262845 107267679 107268133
TCA657388 659287 663383
DPA109537502 109537981 109539467 109540738
SOY115875099 115881569 115885155
ATD109596360 109599451 109609672
CSET123311160 123316160 123321334
AGB108912068 108912966 108913506
LDC111502043 111503945 111509249
NVL108557086 108557670 108558174 108567151
APLN108735767 108740927 108743766
PPYR116163081 116164508 116166831 116171124
OTU111414203 111419426 111427848
BMOR101736061 101741346 101742984
BMAN114247002 114248987 114249440
MSEX115440710 115448364 115450427
DPLKGM_203672 KGM_208641 KGM_214517
BANY112045658 112046889 112047335
PMAC106708978 106709701 106712471
PPOT106100685 106105805 106107976
PXU106117396 106121928 106124397
PRAP110993283 111000541 111003256
ZCE119829569 119830202 119834064
HAW110370155 110374076 110376090
TNL113493550 113496594 113505944
SLIU111353115 111355951 111357626
OFU114349767 114352184 114352413
PXY105388581 105389344 105394758 105394780
API100164685 100167784 100167795
DNX107161983 107163206 107174001
AGS114120422 114122118 114131437
RMD113556372 113556757 113560806
BTAB109030556 109034604 109035894
DCI103505288 103516295
CLEC106672266 106673227 106674189
HHAL106678975 106679847 106681771
NLU111049614 111055975 111056777
PHUPhum_PHUM455050 Phum_PHUM488440 Phum_PHUM490250
FOC113203130 113211216 113216463
ZNE110827878 110831848 110831980
CSEC111866806 111866884 111872464
FCD110846056 110847436 110859221
DPXDAPPUDRAFT_218850 DAPPUDRAFT_304945 DAPPUDRAFT_307216
DMK116933567 116933752 116934955
PVM113802032 113819647 113819966 113824640
PJA122245774 122263365 122264403
PCHN125027164 125032085 125036743
HAME121864489 121869478 121874923
PCLA123758376 123762596 123769944
PTRU123501535 123501664 123501943
HAZT108665016 108671440 108677950
EAF111696021 111700775 111718430
ISCIscW_ISCW000326 IscW_ISCW011872 IscW_ISCW021392
DSV119457583 119461512 119461515 119464578
RSAN119374094 119396015 119402119
RMP119165413 119167082 119174327
VDE111246358 111248228 111251828
VJA111265185 111268050 111268602
TUT107361403 107366477 107367244 107371591 107371607
DPTE113789732 113795874 113796479
DFR124491080 124491918 124498786
CSCU111630346 111639700 111639960
PTEP107437365 107450714 107455291
SDM118185853 118192709 118200215
CELCELE_F12F6.3(rib-1) CELE_K01G5.6(rib-2)
CBRCBG_18318(Cbr-rib-2)
BMYBM_BM10349(Bma-rib-2) BM_BM3322(Bma-rib-1) BM_BM4732(Bm4732)
LOALOAG_05822 LOAG_07208 LOAG_08703
NAINECAME_01875 NECAME_03535 NECAME_04728
TSPTsp_02636 Tsp_04346
HROHELRODRAFT_119925 HELRODRAFT_186788 HELRODRAFT_83262
LGILOTGIDRAFT_124977 LOTGIDRAFT_137450 LOTGIDRAFT_199269
PCAN112561913 112562383 112565643
BGT106055537 106058680 106070127
GAE121372488 121376972 121377129
HRF124114597 124138230 124149462
HRJ124256095 124270731 124276925 124282773
CRG105324710 105325442 105341576 117690299
MYI110442614 110443488 110451580
PMAX117316492 117317139 117326975
MMER123539606 123540528 123552181
OBI106867259 106868410 106873155 106874784
OSN115223438 115223625 115223691 115223714 115230884 115231411
LAK106152057 106155252 106175986 106181741
SMMSmp_073220 Smp_146320.1 Smp_172060
SHXMS3_0016615
OVIT265_05866 T265_05932 T265_06473 T265_07036
EGLEGR_02934 EGR_06675 EGR_08543
NVE5504655 5505005 5510789
EPA110234330 110240280 110241454 110241460
ATEN116293188 116298104 116307177
ADF107336652 107336815 107341811 107358713
AMIL114957442 114961284 114970312
PDAM113666284 113669827 113677366
SPIS111325684 111326534 111336973
DGT114527489 114528496 114537265
XEN124438051 124456299 124457859
HMG100208615 100212044 100212160
TADTRIADDRAFT_1026 TRIADDRAFT_24279
AQU100636774 100640795 100641175
SMOSELMODRAFT_23945 SELMODRAFT_24030
PPP112279610 112280248 112280650 112281934 112282628 112290993 112294684
CCPCHC_T00009229001
FGRFGSG_12261
FPUFPSE_06328
FVRFVEG_11257
FOXFOXG_13828
NHENECHADRAFT_84083
MBRMONBRDRAFT_25145
SREPTSG_07269 PTSG_09573 PTSG_10121
ACANACA1_335890 ACA1_393390
PTIPHATRDRAFT_11259
FCYFRACYDRAFT_160829 FRACYDRAFT_234481
TPSTHAPSDRAFT_263396
GTTGUITHDRAFT_143314
 » show all
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 98:7176-81 (2001)
DOI:10.1073/pnas.131188498
  Sequence
[hsa:2134]
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
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 1:282-6 (2000)
DOI:10.1093/embo-reports/kvd045
  Sequence
[bta:281151]
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 273:26265-8 (1998)
DOI:10.1074/jbc.273.41.26265
  Sequence
[bta:281151]
Other DBs
ExplorEnz - The Enzyme Database: 2.4.1.224
IUBMB Enzyme Nomenclature: 2.4.1.224
ExPASy - ENZYME nomenclature database: 2.4.1.224
BRENDA, the Enzyme Database: 2.4.1.224
CAS: 336193-98-7
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