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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)
CGE: 100689334(Ext1) 100751585(Ext2)
NGI: 103738135(Ext1) 103751554(Ext2)
HGL: 101706257(Ext1) 101715817(Ext2)
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)
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)
LAV: 100656016(EXT2) 100657623(EXT1)
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)
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)
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)
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)
LCM: 102353460(EXT1) 102354369 102354854(EXTL1) 102363354(EXT2)
DME: Dmel_CG10117(ttv) Dmel_CG8433(sotv)
DSI: Dsimw501_GD11060(Dsim_GD11060) Dsimw501_GD25567(Dsim_GD25567)
OBO: 105287638
NVI: 100120177
CEL: CELE_F12F6.3(rib-1)
CBR: CBG06125(Cbr-rib-1)
TSP: Tsp_04346
EGL: EGR_02934
RCN: 112167995
PMUM: 103333289
PDA: 103718232
EGU: 105051507
CRE: CHLREDRAFT_144608(ELG2)
 » 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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