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
HSA: 2131(EXT1) 2132(EXT2) 2134(EXTL1) 2137(EXTL3)
PTR: 107976420(EXT1) 451140(EXT2) 464086(EXTL3) 469238(EXTL1)
PPS: 100975712(EXT2) 100979888(EXTL1) 100984535(EXTL3) 100985695(EXT1)
GGO: 101130563(EXT2) 101144361(EXTL3) 101149310(EXTL1) 109025298(EXT1)
PON: 100172231(EXTL1) 100172450(EXT1) 100436160(EXT2) 100458560(EXTL3)
NLE: 100590557(EXTL3) 100597702(EXT1) 100604250(EXT2) 105738035(EXTL1)
MCC: 100426593(EXTL3) 701488(EXT1) 713664(EXTL1) 715975(EXT2)
MCF: 101865433(EXTL3) 101926231(EXT1) 102125916(EXTL1) 102136104(EXT2)
CSAB: 103215549(EXTL3) 103225338(EXTL1) 103236980 103237338(EXT1)
RRO: 104656082(EXTL1) 104658897(EXT1) 104668244(EXTL3) 104673882(EXT2)
RBB: 108518038(EXTL1) 108519255(EXT2) 108536176(EXT1) 108537365(EXTL3)
CJC: 100386964(EXT1) 100400452(EXTL3) 100400679(EXT2) 100412852(EXTL1)
SBQ: 101037632(EXTL3) 101039408(EXT2) 101053138(EXTL1) 101053444(EXT1)
MMU: 14042(Ext1) 14043(Ext2) 54616(Extl3) 56219(Extl1)
MCAL: 110292428(Extl1) 110309303(Extl3) 110310432(Ext1) 110311448(Ext2)
MPAH: 110317782(Ext2) 110323450(Extl1) 110325330(Extl3) 110334870(Ext1)
RNO: 299907(Ext1) 311215(Ext2) 313610(Extl1) 56819(Extl3)
MUN: 110540376(Ext2) 110552198(Extl1) 110559132(Extl3) 110559447 110559448
CGE: 100689334(Ext1) 100751585(Ext2) 100751999(Extl3) 100770000(Extl1)
NGI: 103729989(Extl3) 103737100(Extl1) 103738135(Ext1) 103751554(Ext2)
HGL: 101697847(Extl3) 101705292(Extl1) 101706257(Ext1) 101715817(Ext2)
OCU: 100343697(EXT2) 100347418(EXTL1) 100348721(EXTL3) 100351235(EXT1)
TUP: 102467803(EXT1) 102472563(EXTL3) 102487057(EXT2) 102499532(EXTL1)
CFA: 475989(EXT2) 482024(EXT1) 486091(EXTL3) 487363(EXTL1)
VVP: 112915941(EXT1) 112924118(EXT2) 112926771(EXTL3) 112931669(EXTL1)
AML: 100465258(EXT1) 100469588(EXTL3) 100482562(EXTL1) 105242037(EXT2)
UMR: 103661582(EXT1) 103666523(EXTL1) 103672344(EXT2) 103675952(EXTL3)
UAH: 113252791(EXT1) 113265011(EXT2) 113268473(EXTL1) 113269941(EXTL3)
ORO: 101366478(EXT2) 101377818(EXTL3) 101379569(EXTL1) 101382133(EXT1)
FCA: 101081362(EXT1) 101083620(EXT2) 101083693(EXTL1) 101095491(EXTL3)
PTG: 102961650(EXTL3) 102962317(EXTL1) 102964333(EXT2) 102968916(EXT1)
PPAD: 109256806(EXTL1) 109262363(EXTL3) 109273237(EXT1) 109279210 109279211(EXT2)
AJU: 106975536(EXT1) 106977618(EXTL1) 106978049(EXT2) 106983017(EXTL3)
BTA: 281151(EXT2) 511060(EXTL1) 538602(EXT1) 783970(EXTL3)
BOM: 102267135(EXTL1) 102273632(EXTL3) 102275740(EXT2) 102280308(EXT1)
BIU: 109562800(EXTL3) 109568471(EXT1) 109569634(EXT2) 109573580(EXTL1)
BBUB: 102389161(EXT2) 102398616(EXTL3) 102407802(EXT1) 102415224(EXTL1)
CHX: 102178172(EXTL3) 102182004(EXTL1) 102182243(EXT2) 102182809(EXT1)
OAS: 101108814(EXTL3) 101110867(EXT2) 101113394(EXTL1) 101114877(EXT1)
SSC: 100152703(EXTL3) 100157655(EXT1) 100522362(EXT2) 100623848(EXTL1)
CFR: 102507432(EXTL3) 102508456(EXT1) 102514905(EXT2) 102521830(EXTL1)
CDK: 105096148(EXT1) 105100672(EXTL1) 105102072(EXTL3) 105106838(EXT2)
BACU: 102999728(EXT2) 103004222(EXTL1) 103009480(EXTL3) 103019432(EXT1)
LVE: 103068452(EXTL1) 103078702(EXTL3) 103078738(EXT1) 103091009(EXT2)
OOR: 101280958(EXT2) 101281062(EXTL1) 101286194(EXTL3) 101286251(EXT1)
DLE: 111163234(EXTL1) 111165183(EXT1) 111184011(EXT2) 111185386(EXTL3)
PCAD: 102979294 102980097(EXT1) 102983199(EXTL1) 102995934(EXTL3)
ECB: 100054778(EXTL3) 100055879(EXT2) 100056983(EXT1) 100071242(EXTL1)
EPZ: 103549478(EXT1) 103556459(EXT2) 103556774(EXTL1) 103567372(EXTL3)
EAI: 106832529(EXTL3) 106839736 106839934(EXTL1) 106843971(EXT2)
MYB: 102238781(EXT2) 102244714(EXT1) 102246778(EXTL1) 102250952(EXTL3)
MYD: 102761567(EXTL1) 102764127(EXTL3) 102765321(EXT1) 102767712(EXT2)
MNA: 107525491(EXT2) 107533185(EXT1) 107537770(EXTL3) 107541891(EXTL1)
HAI: 109379533(EXTL3) 109382533 109386781(EXTL1) 109391972(EXT2)
DRO: 112299237(EXTL1) 112303473(EXT2) 112312602(EXTL3) 112315333(EXT1)
PALE: 102889624(EXTL3) 102890634(EXT2) 102891150(EXT1) 102894796(EXTL1)
RAY: 107502529(EXTL1) 107507149(EXT1) 107510781(EXTL3) 107511887(EXT2)
MJV: 108386769 108391555(EXTL3) 108394755(EXT2) 108398779(EXTL1) 108399086(EXT1) 108399399
LAV: 100656016(EXT2) 100657623(EXT1) 100666182(EXTL3) 100673302(EXTL1)
MDO: 100010947(EXT2) 100015039(EXTL1) 100025396(EXT1) 100030828(EXTL3)
SHR: 100918977(EXTL1) 100919560(EXTL3) 100929895(EXT1) 105750949(EXT2)
PCW: 110200303(EXT1) 110206108(EXTL1) 110209977(EXTL3) 110223945(EXT2)
OAA: 100078206(EXT1) 100079286(EXT2) 100087229(EXTL3)
GGA: 100858205(EXT1) 100859367(EXTL1) 422022(EXTL3) 425859(EXT2)
MGP: 100542119(EXT1) 100542317(EXTL3) 100545109 100551486(EXTL1)
CJO: 107310492(EXT1) 107312356(EXTL3) 107314816(EXT2) 107324063(EXTL1)
NMEL: 110387476(EXTL1) 110395119(EXT1) 110396622(EXTL3) 110401311(EXT2)
APLA: 101790687(EXT2) 101791181(EXTL3) 101792373(EXT1)
ACYG: 106037911(EXTL1) 106038151(EXTL3) 106038456(EXT1) 106044125(EXT2)
TGU: 100227900(EXT1) 100228076(EXTL3) 100231605(EXT2) 115498288(EXTL1)
LSR: 110472037(EXT2) 110472893(EXTL1) 110476418(EXTL3) 110479282(EXT1)
SCAN: 103814231(EXT1) 103821407(EXTL3) 103823579(EXTL1) 103826716(EXT2)
GFR: 102035022(EXTL1) 102040196(EXT2) 102041533(EXTL3) 102043948(EXT1)
FAB: 101814666(EXT2) 101816822(EXT1) 101818645(EXTL3) 101818678(EXTL1)
PHI: 102099981(EXT1) 102105383(EXTL3) 102107359(EXT2) 102111356(EXTL1)
PMAJ: 107200137(EXT1) 107201996(EXTL3) 107205846(EXT2) 107213996(EXTL1)
CCAE: 111925064(EXT1) 111927356(EXTL3) 111930141(EXT2)
CCW: 104692676(EXT1) 104694746(EXTL1) 104695274(EXT2) 104696886(EXTL3)
ETL: 114057979(EXT1) 114064860(EXT2) 114068426(EXTL3) 114070342(EXTL1)
FPG: 101917228(EXTL3) 101919384(EXT1) 101922176(EXTL1) 101923547(EXT2)
FCH: 102049940(EXT1) 102054426(EXT2) 102056733(EXTL3) 102056866(EXTL1)
CLV: 102084082(EXTL1) 102085197(EXT2) 102088676(EXTL3) 102091811(EXT1)
EGZ: 104123693(EXT1) 104127251(EXTL3) 104134053(EXT2) 104134955(EXTL1)
NNI: 104014701(EXTL3) 104017847(EXTL1) 104018021(EXT1) 104019563(EXT2)
ACUN: 113476897(EXT1) 113478245(EXTL3) 113480641(EXT2) 113488189(EXTL1)
PADL: 103913240(EXTL3) 103914958(EXT1) 103921685(EXT2)
ASN: 102370729(EXT1) 102376854(EXTL1) 102377426(EXTL3) 102379048(EXT2)
AMJ: 102560415(EXT1) 102562202(EXT2) 102575775(EXTL3) 102575942(EXTL1)
PSS: 102444477(EXT1) 102446763(EXT2) 102462577(EXTL3)
CMY: 102929859 102938478(EXT2) 102939595(EXT1) 102945736(EXTL3)
CPIC: 101935250(EXTL3) 101946173(EXT1) 101949276(EXT2) 101953823(EXTL1) 103307378
ACS: 100553800(extl1) 100555771(ext2) 100557117(extl3) 100567014(ext1)
PVT: 110070203(EXTL3) 110076972(EXT1) 110081549(EXT2) 110084159(EXTL1)
PBI: 103048904 103054708 103060383(EXTL3) 103062807(EXT2) 103067611(EXTL1)
PMUR: 107284865(EXTL3) 107292260 107295666(EXTL1) 107302595(EXT2)
TSR: 106538947 106539688(EXTL1) 106541899(EXTL3) 106545012(EXT2)
PMUA: 114594927(EXTL3) 114596253(EXT2) 114601312(EXT1) 114601546(EXTL1)
GJA: 107106370(EXTL3) 107110827(EXTL1) 107113379 107122615(EXT2)
XLA: 380140(ext2.L) 398214(ext1.L) 398750(ext2.S) 399115(ext1.S) 444275 447542(extl3.L)
XTR: 100151709(ext2) 448393(ext1) 493398(extl3)
NPR: 108786158(EXT2) 108790563(EXT1) 108802201(EXTL3)
DRE: 101885478 493780(ext2) 493783(extl3) 497279(ext1a) 497280(ext1b) 497281(ext1c)
IPU: 108256617 108257122(extl1) 108257140(ext1) 108257719(extl3) 108271344(ext2) 108275270
LCM: 102353460(EXT1) 102354369 102354854(EXTL1) 102361988(EXTL3) 102363354(EXT2)
DME: Dmel_CG10117(ttv) Dmel_CG15110(botv) Dmel_CG8433(sotv)
DSI: Dsimw501_GD11060(Dsim_GD11060) Dsimw501_GD11429(Dsim_GD11429) Dsimw501_GD25567(Dsim_GD25567)
CEL: CELE_F12F6.3(rib-1) CELE_K01G5.6(rib-2)
CBR: CBG06125(Cbr-rib-1) CBG18318(Cbr-rib-2)
 » 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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