KEGG   ENZYME: 2.4.1.225Help
Entry
EC 2.4.1.225                Enzyme                                 

Name
N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase;
N-acetylglucosaminylproteoglycan beta-1,4-glucuronyltransferase;
heparan glucuronyltransferase II
Class
Transferases;
Glycosyltransferases;
Hexosyltransferases
BRITE hierarchy
Sysname
UDP-alpha-D-glucuronate:N-acetyl-alpha-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-proteoglycan 4-beta-glucuronosyltransferase
Reaction(IUBMB)
UDP-alpha-D-glucuronate + N-acetyl-alpha-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-proteoglycan = UDP + beta-D-glucuronosyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-proteoglycan [RN:R07335]
Reaction(KEGG)
Substrate
UDP-alpha-D-glucuronate [CPD:C00167];
N-acetyl-alpha-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-proteoglycan
Product
UDP [CPD:C00015];
beta-D-glucuronosyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-proteoglycan
Comment
Involved in the biosynthesis of heparin and heparan sulfate. Some forms of the human enzyme (particularly the enzyme complex encoded by the EXT1 and EXT2 genes) act as bifunctional glycosyltransferases, which also have the glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase (EC 2.4.1.224) activity required for the synthesis of the heparan sulfate disaccharide repeats.
History
EC 2.4.1.225 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
Genes
HSA: 2131(EXT1) 2132(EXT2)
PTR: 107976420(EXT1) 451140(EXT2)
PPS: 100975712(EXT2) 100985695(EXT1)
GGO: 101130563(EXT2) 109025298(EXT1)
PON: 100172450(EXT1) 100436160(EXT2)
NLE: 100597702(EXT1) 100604250(EXT2)
MCC: 701488(EXT1) 715975(EXT2)
MCF: 101926231(EXT1) 102136104(EXT2)
CSAB: 103236980 103237338(EXT1)
RRO: 104658897(EXT1) 104673882(EXT2)
RBB: 108519255(EXT2) 108536176(EXT1)
CJC: 100386964(EXT1) 100400679(EXT2)
SBQ: 101039408(EXT2) 101053444(EXT1)
MMU: 14042(Ext1) 14043(Ext2)
MCAL: 110310432(Ext1) 110311448(Ext2)
MPAH: 110317782(Ext2) 110334870(Ext1)
RNO: 299907(Ext1) 311215(Ext2)
MUN: 110540376(Ext2) 110554144 110559447 110559448
CGE: 100689334(Ext1) 100751585(Ext2)
NGI: 103738135(Ext1) 103751554(Ext2)
HGL: 101706257(Ext1) 101715817(Ext2)
CCAN: 109686416 109686418 109686420 109701414 109702003
OCU: 100343697(EXT2) 100351235(EXT1)
TUP: 102467803(EXT1) 102487057(EXT2)
CFA: 475989(EXT2) 482024(EXT1)
VVP: 112915941(EXT1) 112924118(EXT2)
AML: 100465258(EXT1) 100476265 105242037(EXT2)
UMR: 103661582(EXT1) 103672344(EXT2)
UAH: 113252791(EXT1) 113265011(EXT2)
ORO: 101366478(EXT2) 101382133(EXT1)
ELK: 111146555 111150031
FCA: 101081362(EXT1) 101083620(EXT2)
PTG: 102964333(EXT2) 102968916(EXT1)
PPAD: 109273237(EXT1) 109279210 109279211(EXT2)
AJU: 106975536(EXT1) 106978049(EXT2)
BTA: 281151(EXT2) 538602(EXT1)
BOM: 102275740(EXT2) 102280308(EXT1)
BIU: 109568471(EXT1) 109569634(EXT2)
BBUB: 102389161(EXT2) 102407802(EXT1)
CHX: 102182243(EXT2) 102182809(EXT1)
OAS: 101110867(EXT2) 101114877(EXT1)
SSC: 100157655(EXT1) 100522362(EXT2)
CFR: 102508456(EXT1) 102514645(EXT2)
CDK: 105096148(EXT1) 105106838(EXT2)
BACU: 102999728(EXT2) 103019432(EXT1)
LVE: 103078738(EXT1) 103091009(EXT2)
OOR: 101280958(EXT2) 101286251(EXT1)
DLE: 111165183(EXT1) 111184011(EXT2)
PCAD: 102979294 102980097(EXT1)
ECB: 100055879(EXT2) 100056983(EXT1)
EPZ: 103549478(EXT1) 103556459(EXT2)
EAI: 106839736 106843971(EXT2)
MYB: 102238781(EXT2) 102244714(EXT1)
MYD: 102765321(EXT1) 102767712(EXT2)
MNA: 107525491(EXT2) 107533185(EXT1)
HAI: 109382533 109391972(EXT2)
DRO: 112303473(EXT2) 112315333(EXT1)
PALE: 102890634(EXT2) 102891150(EXT1)
RAY: 107507149(EXT1) 107511887(EXT2)
MJV: 108386769 108394755(EXT2) 108399086(EXT1) 108399399
LAV: 100656016(EXT2) 100657623(EXT1)
TMU: 101361975 101362012
MDO: 100010947(EXT2) 100025396(EXT1)
SHR: 100929895(EXT1) 105750949(EXT2)
PCW: 110200303(EXT1) 110223945(EXT2)
OAA: 100078206(EXT1) 100079286(EXT2)
GGA: 100858205(EXT1) 100859367(EXTL1) 425859(EXT2)
MGP: 100542119(EXT1) 100545109 100551486(EXTL1)
CJO: 107310492(EXT1) 107314816(EXT2) 107324063(EXTL1)
NMEL: 110387476(EXTL1) 110395119(EXT1) 110401311(EXT2)
APLA: 101790687(EXT2) 101792373(EXT1)
ACYG: 106037911(EXTL1) 106038456(EXT1) 106044125(EXT2)
TGU: 100227900(EXT1) 100231605(EXT2)
LSR: 110472037(EXT2) 110472893(EXTL1) 110479282(EXT1)
SCAN: 103814231(EXT1) 103823579(EXTL1) 103826716(EXT2)
GFR: 102035022(EXTL1) 102040196(EXT2) 102043948(EXT1)
FAB: 101814666(EXT2) 101816822(EXT1) 101818678(EXTL1)
PHI: 102099981(EXT1) 102107359(EXT2) 102111356(EXTL1)
PMAJ: 107200137(EXT1) 107205846(EXT2) 107213996(EXTL1)
CCAE: 111922012 111925064(EXT1) 111930141(EXT2) 111944129
CCW: 104692676(EXT1) 104694746(EXTL1) 104695274(EXT2)
ETL: 114057979(EXT1) 114064860(EXT2) 114070342(EXTL1)
FPG: 101919384(EXT1) 101922176(EXTL1) 101923547(EXT2)
FCH: 102049940(EXT1) 102054426(EXT2) 102056866(EXTL1)
CLV: 102084082(EXTL1) 102085197(EXT2) 102091811(EXT1)
EGZ: 104123693(EXT1) 104134053(EXT2) 104134955(EXTL1)
NNI: 104017847(EXTL1) 104018021(EXT1) 104019563(EXT2)
ACUN: 113476897(EXT1) 113480641(EXT2) 113488189(EXTL1)
PADL: 103914958(EXT1) 103921685(EXT2)
AAM: 106485009 106496271(EXT1) 106498294(EXT2) 106499622
ASN: 102370729(EXT1) 102376854(EXTL1) 102379048(EXT2)
AMJ: 102560415(EXT1) 102562202(EXT2) 102575942(EXTL1)
PSS: 102444477(EXT1) 102446763(EXT2)
CMY: 102929859 102938478(EXT2) 102939595(EXT1)
CPIC: 101946173(EXT1) 101949276(EXT2) 101953823(EXTL1) 103307378
ACS: 100553800(extl1) 100555771(ext2) 100567014(ext1)
PVT: 110076972(EXT1) 110081549(EXT2) 110084159(EXTL1)
PBI: 103048904 103053410 103054708 103062807(EXT2) 103067611(EXTL1)
PMUR: 107292260 107295666(EXTL1) 107302595(EXT2)
TSR: 106538947 106539688(EXTL1) 106545012(EXT2)
PMUA: 114596253(EXT2) 114601312(EXT1) 114601546(EXTL1)
GJA: 107110827(EXTL1) 107113379 107122615(EXT2)
XLA: 380140(ext2.L) 398214(ext1.L) 398750(ext2.S) 399115(ext1.S)
XTR: 100151709(ext2) 448393(ext1)
NPR: 108786158(EXT2) 108790563(EXT1)
DRE: 493780(ext2) 497279(ext1a) 497280(ext1b) 497281(ext1c)
SRX: 107706235(extl1) 107706241 107711539 107715577 107727350 107733302 107742222 107750554 107754264 107754269 107754691
SANH: 107654722 107654729 107659874 107671371 107671458 107679666 107683605 107685778 107688159(ext2) 107688215 107688378 107696533
SGH: 107548414 107552442 107558762 107562300 107575740 107586005 107592048(extl1) 107592050 107592164 107592487 107592940 107592945 107593432
CCAR: 109050530 109050531 109056569 109059721 109072430 109074535(ext2) 109075419 109075422 109075442 109075920 109093562 109102354 109106263 109109197 109109198 109109965 109111718 109113794
IPU: 108256617 108257122(extl1) 108257140(ext1) 108271344(ext2) 108275270
PHYP: 113529295 113532714 113535947 113535988 113536365(ext2)
AMEX: 103026919 103031914 103040053 103046596(ext2) 103047171 111197650
EEE: 113575759(ext2) 113578814 113588018(ext1) 113591568 113591687
TRU: 101065654 101066048(ext2) 101072426 101072822 101075972
TNG: GSTEN00017827G001 GSTEN00032917G001 GSTEN00032935G001 GSTEN00035381G001 GSTEN00035475G001 GSTEN00037399G001 GSTEN00037752G001 GSTEN00038113G001
LCO: 104919199(ext2) 104926220 104931682 104932462 104937142
NCC: 104945394 104946866 104947131 104949435 104961795(ext1) 104963025(ext2)
MZE: 101464589 101473582(ext2) 101479111 101482519 101486492 112433521
ONL: 100695641(ext2) 100700775 100705583 100707723 109194193
OLA: 101163398(ext2) 101164181 101167217 101169374
XMA: 102217307 102217354 102230266 102234444(ext2) 102237506
XCO: 114141516 114142777(ext2) 114155469 114156156 114159116
PRET: 103457265 103462432(ext2) 103472886(ext1) 103473086 103478346
CVG: 107082854 107085591(ext2) 107086154 107089629(ext1) 107094248 107094935
NFU: 107377760 107378775 107381443(ext2) 107394502 107394966
KMR: 108228674(ext2) 108232308 108243943 108244864 108249023 108250176
ALIM: 106511501 106514225(ext1) 106527530 106530767(ext2) 106532427 106535689
AOCE: 111563665 111564587 111569354 111571373 111574611(ext2) 111580481 111588665 111589114(ext1)
CSEM: 103378751(ext2) 103381137 103388612(ext1) 103393992 103394040 103394083
POV: 109632177(ext1) 109636768(ext2) 109639965 109639984 109642456 109642534 109643173 109644500
LCF: 108888686 108889564 108889565 108889668(ext2) 108889677 108891202
SDU: 111222995 111223271 111227735 111237872 111238419(ext2)
SLAL: 111654812(ext2) 111657821 111658101 111671857 111671874 111672565
HCQ: 109514394(ext2) 109516322 109518055 109518060 109523479 109529689(ext1)
BPEC: 110153759 110156338 110171534 110172856(ext2) 110173279
MALB: 109952487 109958190 109958643 109958685 109965798(ext2) 109968834
SASA: 100306779(ext1c) 100380322(ext2) 106570050 106571404 106588496 106588504 106591521 106593102 106593181 106594121 106597672 106598735 106599255 106606169 106606176 106607809
OTW: 112217339 112217608 112229926 112229932 112247034 112250171 112264222 112264884 112264892
SALP: 111954432 111955132 111955294 111955814 111955850 111969484 111972462
ELS: 105005807 105017786 105022258(ext2) 105023503 105027867
SFM: 108922050(ext1) 108928562 108931808 108934682(ext2) 108940524
PKI: 111836091 111840708(ext2) 111840863 111843133 111843930 111843931 111844061 111854291
LCM: 102353460(EXT1) 102354369 102354854(EXTL1) 102363354(EXT2)
CMK: 103174976(ext2) 103179627 103184956 103185802
RTP: 109910890(ext2) 109914394 109917722 109930524 109932300
BFO: BRAFLDRAFT_153069 BRAFLDRAFT_198850 BRAFLDRAFT_198883 BRAFLDRAFT_64151(Slc6a15) BRAFLDRAFT_91377
CIN: 100176235 100185120
SPU: 100893938 577995
APLC: 110981069 110986223
SKO: 100313593 100372539 102809744
DME: Dmel_CG10117(ttv) Dmel_CG8433(sotv)
DER: 6548926 6549168
DSE: Dsec_GM19618 Dsec_GM20090 Dsec_GM21554
DSI: Dsimw501_GD11060(Dsim_GD11060) Dsimw501_GD25567(Dsim_GD25567)
DYA: Dyak_GE13596 Dyak_GE14097
DAN: Dana_GF13049 Dana_GF13249
DSR: 110177800 110178267
DPO: Dpse_GA10084 Dpse_GA17244
DPE: 6590702 6590872
DMN: 108159562 108159662
DWI: 6640721 6640835
DGR: Dgri_GH20211 Dgri_GH20787
DMO: Dmoj_GI20737 Dmoj_GI20872
DAZ: 108615183 108615817
DNV: 108652943 115564114 115564922
DHE: 111600127 111602550
DVI: Dvir_GJ20474 Dvir_GJ20607
MDE: 101888783 101894872 101901385
LCQ: 111677016 111677307
AGA: AgaP_AGAP000081 AgaP_AGAP010388
AAG: 5566768 5578353
AALB: 109397881 109411435 109416900 109428863 115254192
CQU: CpipJ_CPIJ009765 CpipJ_CPIJ012734
AME: 408293 726216
BIM: 100741317 100744382
BTER: 100643223 100650849
CCAL: 108622677 108627221
OBB: 114871565 114871663 114880970
SOC: 105195910 105198634
MPHA: 105835292 105839250
AEC: 105150811 105153382
ACEP: 105619491 105627211
PBAR: 105425236 105427034
VEM: 105564271 105564746
HST: 105181013 105188235
DQU: 106742285 106750617
CFO: 105253055 105253243
LHU: 105673709 105675813
PGC: 109854839 109863645
OBO: 105287638
PCF: 106790860 106791047
NVI: 100120177
CSOL: 105364815 105366129
MDL: 103578696 103580382
TCA: 657388 659287
DPA: 109537981 109539467 109540738 109540739
ATD: 109599451 109609672
NVL: 108557086 108557670 108558174
BMOR: 101736061 101741346 105841885
BMAN: 114247002 114249440
DPL: KGM_203672 KGM_214517
PMAC: 106708978 106709701
PRAP: 110993283 111003256
HAW: 110374076 110376090
TNL: 113496594 113505944
PXY: 105389344 105393835 105394758 105394780
API: 100164685 100167795
DNX: 107161983 107174001
AGS: 114120422 114131437
RMD: 113556757 113560806
BTAB: 109034604 109035894
CLEC: 106673227 106674189
PHU: Phum_PHUM455050 Phum_PHUM488440
ZNE: 110827878 110831848
FCD: 110847436 110859221
DPX: DAPPUDRAFT_304945 DAPPUDRAFT_307216
PVM: 113802032 113819966 113824640
ISC: IscW_ISCW000326 IscW_ISCW021389 IscW_ISCW021390 IscW_ISCW021392
TUT: 107361403 107367244 107371591 107371607
DPTE: 113789732 113796479
CSCU: 111617038 111629514 111630346 111639960
PTEP: 107437365 107450714
CEL: CELE_F12F6.3(rib-1)
CBR: CBG06125(Cbr-rib-1)
BMY: Bm1_15685 Bm1_26565
LOA: LOAG_05822 LOAG_08703
NAI: NECAME_01875 NECAME_01876 NECAME_04728
TSP: Tsp_04346
HRO: HELRODRAFT_119925 HELRODRAFT_186788
LGI: LOTGIDRAFT_124977 LOTGIDRAFT_199269
PCAN: 112561913 112565643
CRG: 105324710 105325442
MYI: 110443488 110451580
OBI: 106867259 106868410 106869261
LAK: 106155252 106175986 106181741
SMM: Smp_146320.1 Smp_172060
SHX: MS3_03971 MS3_06951
OVI: T265_05866 T265_05932
EGL: EGR_02934
NVE: NEMVE_v1g168451 NEMVE_v1g173450
EPA: 110240280 110241454 110241460
ADF: 107336652 107336815 107358713
AMIL: 114957442 114961284
PDAM: 113666284 113669827
SPIS: 111319796 111326534 111336973
DGT: 114528496 114537265
HMG: 100208615 100212044
TAD: TRIADDRAFT_1026 TRIADDRAFT_24279
AQU: 100636774 100641175
RCN: 112167995
PMUM: 103333289
PDA: 103718232
EGU: 105051507
SMO: SELMODRAFT_23945 SELMODRAFT_24030
PPP: 112279610 112280248 112280650 112281934 112282628 112290993 112294684
CRE: CHLREDRAFT_144608(ELG2)
BPG: Bathy19g00100
GSL: Gasu_04130
NHE: NECHADRAFT_84083
SRE: PTSG_07269 PTSG_07754 PTSG_10121
ACAN: ACA1_335890 ACA1_393390
PTI: PHATRDRAFT_11259
FCY: FRACYDRAFT_234481
TPS: THAPSDRAFT_263396
AAF: AURANDRAFT_39371
 » show all
Taxonomy
Reference
1  [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
2  [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: 2.4.1.225
IUBMB Enzyme Nomenclature: 2.4.1.225
ExPASy - ENZYME nomenclature database: 2.4.1.225
BRENDA, the Enzyme Database: 2.4.1.225
CAS: 145539-84-0

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Pathway (5)   
   KEGG PATHWAY (4)   
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Chemical substance (10)   
   KEGG COMPOUND (4)   
   KEGG GLYCAN (6)   
Chemical reaction (5)   
   KEGG REACTION (3)   
   KEGG RCLASS (2)   
Gene (4724)   
   KEGG ORTHOLOGY (2)   
   KEGG GENES (791)   
   KEGG MGENES (770)   
   RefGene (3161)   
Protein sequence (83)   
   UniProt (43)   
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   RefSeq(pep) (23)   
   PMD (2)   
DNA sequence (46)   
   RefSeq(nuc) (29)   
   GenBank (12)   
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