KEGG   ORTHOLOGY: K10158
Entry
K10158                      KO                                     
Symbol
B3GAT3
Name
galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase 3 [EC:2.4.1.135]
Pathway
map00532  Glycosaminoglycan biosynthesis - chondroitin sulfate / dermatan sulfate
map00534  Glycosaminoglycan biosynthesis - heparan sulfate / heparin
map01100  Metabolic pathways
Module
M00057  Glycosaminoglycan biosynthesis, linkage tetrasaccharide
Reaction
R05928  
Disease
H01498  Multiple joint dislocations, short stature, craniofacial dysmorphism, and congenital heart defects
Brite
KEGG Orthology (KO) [BR:ko00001]
 09100 Metabolism
  09107 Glycan biosynthesis and metabolism
   00532 Glycosaminoglycan biosynthesis - chondroitin sulfate / dermatan sulfate
    K10158  B3GAT3; galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase 3
   00534 Glycosaminoglycan biosynthesis - heparan sulfate / heparin
    K10158  B3GAT3; galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase 3
 09180 Brite Hierarchies
  09181 Protein families: metabolism
   01003 Glycosyltransferases
    K10158  B3GAT3; galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase 3
Enzymes [BR:ko01000]
 2. Transferases
  2.4  Glycosyltransferases
   2.4.1  Hexosyltransferases
    2.4.1.135  galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase
     K10158  B3GAT3; galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase 3
Glycosyltransferases [BR:ko01003]
 Glycan extension
  O-Glycan
   K10158  B3GAT3; galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase 3
Other DBs
GO: 0015018
CAZy: GT43
Genes
HSA: 26229(B3GAT3)
PTR: 740388(B3GAT3)
PPS: 103783318(B3GAT3)
GGO: 109028750(B3GAT3)
PON: 100458701(B3GAT3)
PPYG: 129008224(B3GAT3)
NLE: 100592146(B3GAT3)
HMH: 116468888(B3GAT3)
SSYN: 129488696(B3GAT3)
MCC: 718744(B3GAT3)
MCF: 101867087(B3GAT3)
MTHB: 126935491
MNI: 105469443(B3GAT3)
CSAB: 103234006(B3GAT3)
CATY: 105577553(B3GAT3)
PANU: 101012905(B3GAT3)
TGE: 112605682(B3GAT3)
MLEU: 105549041(B3GAT3)
RRO: 104679349(B3GAT3)
RBB: 108533582(B3GAT3)
TFN: 117081104(B3GAT3)
CANG: 105512275(B3GAT3)
CJC: 100395367(B3GAT3)
SBQ: 101034661(B3GAT3)
CIMI: 108286267(B3GAT3) 108297652
ANAN: 105718641(B3GAT3)
CSYR: 103264742(B3GAT3)
MMUR: 105878602(B3GAT3)
LCAT: 123641313(B3GAT3)
PCOQ: 105827019(B3GAT3)
OGA: 100948864(B3GAT3)
MMU: 72727(B3gat3)
MCAL: 110285876(B3gat3)
MPAH: 110320357(B3gat3)
RNO: 293722(B3gat3)
MCOC: 116103532(B3gat3)
ANU: 117698980(B3gat3)
MUN: 110557462(B3gat3)
CGE: 100689419(B3gat3)
MAUA: 121141157(B3gat3)
PROB: 127228824(B3gat3)
PLEU: 114683612(B3gat3)
MORG: 121454137(B3gat3)
MFOT: 126498498
AAMP: 119800517(B3gat3)
NGI: 103737681(B3gat3)
HGL: 101717898(B3gat3)
CPOC: 100731926(B3gat3)
CCAN: 109681240(B3gat3)
DORD: 105995546(B3gat3)
DSP: 122105199(B3gat3)
PLOP: 125362763(B3gat3)
NCAR: 124960761
MMMA: 107135919(B3gat3)
OCU: 100350294
OPI: 101521347(B3GAT3)
TUP: 102472891(B3GAT3)
GVR: 103591081(B3GAT3)
CFA: 483785(B3GAT3)
CLUD: 112659540(B3GAT3)
VVP: 112924375(B3GAT3)
VLG: 121501700(B3GAT3)
NPO: 129503214(B3GAT3)
AML: 100467358(B3GAT3)
UMR: 103678187(B3GAT3)
UAH: 113247005(B3GAT3)
UAR: 123789827(B3GAT3)
ELK: 111150202
LLV: 125078491
MPUF: 101693078(B3GAT3)
MNP: 132020037(B3GAT3)
MLK: 131813948(B3GAT3)
NVS: 122913006(B3GAT3)
ORO: 101373124(B3GAT3)
EJU: 114220493(B3GAT3)
ZCA: 113914299(B3GAT3)
MLX: 118015942(B3GAT3)
NSU: 110575899(B3GAT3)
LWW: 102745335
FCA: 101089044(B3GAT3)
PYU: 121023675(B3GAT3)
PCOO: 112851886(B3GAT3)
PBG: 122483892(B3GAT3)
PVIV: 125147078(B3GAT3)
LRUF: 124505796
PTG: 102948861(B3GAT3)
PPAD: 109246589(B3GAT3)
PUC: 125932295
AJU: 106981737
HHV: 120249216(B3GAT3)
BTA: 404162(B3GAT3)
BOM: 102269297(B3GAT3)
BIU: 109554349(B3GAT3)
BBUB: 102399906(B3GAT3)
BBIS: 104998689(B3GAT3)
CHX: 102172516(B3GAT3)
OAS: 101111917(B3GAT3)
BTAX: 128068709(B3GAT3)
ODA: 120874565(B3GAT3)
CCAD: 122430606(B3GAT3)
MBEZ: 129548868(B3GAT3)
SSC: 397642(B3GAT3)
CFR: 102505525(B3GAT3)
CBAI: 105070128(B3GAT3)
CDK: 105091529(B3GAT3)
VPC: 102540773(B3GAT3)
BACU: 103002629(B3GAT3)
BMUS: 118899058(B3GAT3)
LVE: 103078862(B3GAT3)
OOR: 101279944(B3GAT3)
DLE: 111183870(B3GAT3)
PCAD: 102996092(B3GAT3)
PSIU: 116758116(B3GAT3)
NASI: 112402064(B3GAT3)
ECB: 100063587(B3GAT3)
EPZ: 103558932(B3GAT3)
EAI: 106826833(B3GAT3)
MYD: 102752313(B3GAT3)
MMYO: 118648962(B3GAT3)
MLF: 102440153(B3GAT3)
MDT: 132241545(B3GAT3)
PKL: 118707238(B3GAT3)
EFUS: 103303742(B3GAT3)
MNA: 107543104(B3GAT3)
DRO: 112317373(B3GAT3)
SHON: 118991611(B3GAT3)
AJM: 119035322(B3GAT3)
PDIC: 114499679(B3GAT3)
PHAS: 123829591(B3GAT3)
MMF: 118628796(B3GAT3)
PPAM: 129068435(B3GAT3)
HAI: 109384098(B3GAT3)
RFQ: 117031229(B3GAT3)
PALE: 102891455(B3GAT3)
PGIG: 120600844(B3GAT3)
PVP: 105297798(B3GAT3)
RAY: 107501598(B3GAT3)
MJV: 108393625(B3GAT3)
TOD: 119250084(B3GAT3)
SARA: 101554897(B3GAT3)
SETR: 126017693(B3GAT3)
LAV: 100672369(B3GAT3)
TMU: 101344059
ETF: 101664595(B3GAT3)
DNM: 101447746(B3GAT3)
MDO: 100010236(B3GAT3)
GAS: 123251159(B3GAT3) 123254274
SHR: 100935255(B3GAT3)
AFZ: 127540701
PCW: 110195593(B3GAT3)
TVP: 118855543(B3GAT3)
OAA: 100075413(B3GAT3)
APLA: 119715103(B3GAT3)
AFUL: 116501695(B3GAT3)
OTC: 121339074
OMA: 130262857
PHI: 102105592(B3GAT3)
SVG: 106864156
SHAB: 115614604(B3GAT3)
ACHC: 115338325(B3GAT3)
AGEN: 126033863
RTD: 128901816(B3GAT3)
ASN: 102375316(B3GAT3)
AMJ: 102563043(B3GAT3)
PSS: 102460502(B3GAT3)
CMY: 102941550(B3GAT3)
DCC: 119859352(B3GAT3)
CPIC: 101940176(B3GAT3)
CABI: 116835867(B3GAT3)
MRV: 120409148(B3GAT3)
ACS: 100567109(b3gat3)
ASAO: 132764311(B3GAT3)
PVT: 110081006(B3GAT3)
SUND: 121915744(B3GAT3)
PBI: 103057546(B3GAT3)
PMUR: 107292413(B3GAT3)
CTIG: 120316945(B3GAT3)
PGUT: 117658422(B3GAT3)
APRI: 131186408(B3GAT3)
NSS: 113426894(B3GAT3)
VKO: 123032606(B3GAT3)
PMUA: 114586454(B3GAT3)
PRAF: 128403751(B3GAT3)
ZVI: 118075696(B3GAT3)
HCG: 128337095(B3GAT3)
GJA: 107120072(B3GAT3)
STOW: 125441696(B3GAT3)
EMC: 129338582(B3GAT3)
XLA: 398597(b3gat3.L)
XTR: 594895(b3gat3)
NPR: 108794145(B3GAT3)
RTEM: 120917560(B3GAT3)
BBUF: 120980564(B3GAT3)
BGAR: 122920464(B3GAT3)
MUO: 115479566(B3GAT3)
GSH: 117365953(B3GAT3)
DRE: 192334(b3gat3)
SGH: 107563076 107588211(b3gat3)
PTET: 122349711(b3gat3)
LROH: 127168917(b3gat3)
OMC: 131544528(b3gat3)
PPRM: 120470365(b3gat3)
RKG: 130102712(b3gat3)
MAMB: 125264566(b3gat3)
CIDE: 127516343
CERY: 137030995(b3gat3)
TROS: 130552083(b3gat3)
TDW: 130420870(b3gat3)
MANU: 129440780(b3gat3)
IPU: 108268305
IFU: 128610069(b3gat3)
PHYP: 113539858(b3gat3)
SMEO: 124387453(b3gat3)
TFD: 113635141(b3gat3)
TVC: 132848638(b3gat3)
TRN: 134311573(b3gat3)
AMEX: 103028547(b3gat3)
CMAO: 118826263(b3gat3)
EEE: 113579800(b3gat3)
CHAR: 105895533(b3gat3)
TRU: 654312(b3gat3)
TFS: 130522247(b3gat3)
LCO: 104918106(b3gat3)
NCC: 104960743
TBEN: 117487961(b3gat3)
CGOB: 115023909(b3gat3)
PGEO: 117463830(b3gat3)
GACU: 117549424(b3gat3)
EMAC: 134876313(b3gat3)
ELY: 117272641(b3gat3)
EFO: 125884207(b3gat3)
PLEP: 121962326(b3gat3)
SLUC: 116042344(b3gat3)
ECRA: 117934656(b3gat3)
ESP: 116707125(b3gat3)
PFLV: 114545607(b3gat3)
GAT: 120822759(b3gat3)
PPUG: 119223329(b3gat3)
AFB: 129093283(b3gat3)
CLUM: 117748074(b3gat3)
PSWI: 130211536(b3gat3)
MSAM: 119912927(b3gat3)
SCHU: 122869880(b3gat3)
CUD: 121504191(b3gat3)
ALAT: 119027139(b3gat3)
MZE: 101480695(b3gat3)
ONL: 100690672(b3gat3)
OAU: 116317880(b3gat3)
OLA: 100049460(b3gat3)
OML: 112156302(b3gat3)
CSAI: 133419951(b3gat3)
XMA: 102238000(b3gat3)
XCO: 114157307(b3gat3)
XHE: 116733254(b3gat3)
PRET: 103480760(b3gat3)
PFOR: 103153608(b3gat3)
PLAI: 106948298(b3gat3)
PMEI: 106916892(b3gat3)
GAF: 122820549(b3gat3)
PPRL: 129373279(b3gat3)
CVG: 107098300(b3gat3)
CTUL: 119786650(b3gat3)
GMU: 124880029(b3gat3)
NFU: 107377135(b3gat3)
KMR: 108242856(b3gat3)
ALIM: 106524984(b3gat3)
NWH: 119416873(b3gat3)
AOCE: 111577134(b3gat3)
MCEP: 125024038(b3gat3)
CSEM: 103384246(b3gat3)
SSEN: 122766531(b3gat3)
HHIP: 117751990(b3gat3)
HSP: 118102862(b3gat3)
PPLT: 128429912(b3gat3)
SMAU: 118300167(b3gat3)
LCF: 108888787
SDU: 111231304(b3gat3)
SLAL: 111666406(b3gat3)
XGL: 120797325(b3gat3)
HCQ: 109515067(b3gat3)
SSCV: 125976589
SBIA: 133494421(b3gat3)
PEE: 133397935(b3gat3)
PTAO: 133472640(b3gat3)
BPEC: 110156246(b3gat3)
MALB: 109965809(b3gat3)
BSPL: 114846720(b3gat3)
SJO: 128384223(b3gat3)
SASA: 106578098(b3gat3)
STRU: 115194688(b3gat3)
OTW: 112264921(b3gat3)
OMY: 110531396(b3gat3)
OGO: 124008150(b3gat3)
ONE: 115120586(b3gat3)
OKI: 109906305(b3gat3)
OKE: 118368998(b3gat3)
SALP: 112071457(b3gat3)
SNH: 120036529(b3gat3)
CCLU: 121554359(b3gat3)
ELS: 105005687(b3gat3)
SFM: 108925220(b3gat3)
PKI: 111834547(b3gat3)
AANG: 118223369(b3gat3)
PSPA: 121306256(b3gat3)
PSEX: 120539226(b3gat3)
LCM: 102364764(B3GAT3)
CMK: 103171591(b3gat3)
RTP: 109923317(b3gat3)
CPLA: 122543784(b3gat3)
HOC: 132836087(b3gat3)
PMRN: 116948463(B3GAT3)
LRJ: 133352686(B3GAT3)
BFO: 118409812
BBEL: 109470262
SPU: 579023
APLC: 110981956
ARUN: 117288538
AJC: 117116085
SKO: 100376501
DME: Dmel_CG32775(GlcAT-I)
DER: 6550975
DSE: 6612533
DSI: Dsimw501_GD16673(Dsim_GD16673)
DYA: Dyak_GE16334(Dyak_GlcAT-I)
DAN: 6503472
DSR: 110181804
DPO: 4814302
DPE: 6596722
DMN: 108151945
DWI: 6653043
DGR: 6565886
DAZ: 108618593
DNV: 108657103
DHE: 111593289
DVI: 6633777
CCAT: 101459281
BOD: 106614241
BDR: 105234204
AOQ: 129239226
TDA: 119670011
MDE: 101892716
SCAC: 106093241
LCQ: 111678701
LSQ: 119601070
ECOE: 129944456
CLON: 129611387
HIS: 119654294
AGA: 1273161
ACOZ: 120947568
AARA: 120896559
AMER: 121589821
ASTE: 118506269
AFUN: 125775077
AMOU: 128299200
AALI: 118469018
AAG: 5571591
AALB: 109429162
ASUA: 134225285
CPII: 120421952
CNS: 116339531
BCOO: 119078086
AME: 413271
ACER: 108004368
ALAB: 122711206
ADR: 102674586
AFLR: 100867272
BIM: 100741185
BBIF: 117207113
BVK: 117233648
BVAN: 117158960
BTER: 100643292
BAFF: 126927095
BPYO: 122571587
BPAS: 132908722
FVI: 122536136
CCAL: 108624399
OBB: 114878273
OLG: 117607000
MGEN: 117217444
NMEA: 116426277
CGIG: 122402656
SOC: 105199644
MPHA: 105837929
AEC: 105151612
ACEP: 105617474
PBAR: 105430454
VEM: 105570196
HST: 105182746
DQU: 106745968
CFO: 105256002
FEX: 115234003
LHU: 105676258(GlcAT-I)
PGC: 109851697
OBO: 105284906
PCF: 106785162
PFUC: 122525585
VPS: 122633469
VCRB: 124429451
VVE: 124953809
NVI: 100123025(B3gat)
CSOL: 105363217
TPRE: 106654103
LHT: 122510429
LBD: 127279297
MDL: 103579755
CGLO: 123264217
FAS: 105268776
DAM: 107039494
AGIF: 122853074
CINS: 118072025
VCAN: 122416583
CCIN: 107264164
DSM: 124413502
NPT: 124222549
NFB: 124185831
NLO: 107218273
NVG: 124308223
AROA: 105685161
TCA: 663511
DPA: 109545043
SOY: 115886089
AGRG: 126737307
ATD: 109597949
CSET: 123310139
AGB: 108910299
LDC: 111517774
DVV: 114327544
NVL: 108567330
APLN: 108735077
PPYR: 116168143
OTU: 111414430
BMOR: 101743406
BMAN: 114240951
BANY: 112043611
MJU: 123872969
NIQ: 126769613
VCD: 124535575
MCIX: 123661067
PMAC: 106708809
PPOT: 106104355
PXU: 106121601
PRAP: 110998321
PBX: 123715315
PNAP: 125057290
ZCE: 119832897
CCRC: 123697344
LSIN: 126974130
AAGE: 121734848
HAW: 110384556
HZE: 124636255
TNL: 113495713
SLIU: 111356709
OFU: 114353479
ATRA: 106138377
GMW: 113513311
PXY: 105394656
PGW: 126373176
LGLY: 125235267
CFEL: 113389660
API: 100159562
DNX: 107164038
AGS: 114130210
RMD: 113553674
ACOO: 126834851
DVT: 126904586
BTAB: 109032291
DCI: 103521445
CLEC: 106661064
HHAL: 106679510
NLU: 111056175
FOC: 113204078
TPAL: 117641435
ZNE: 110829492
CSEC: 111865679
SGRE: 126267164
BROR: 134535136
IEL: 124153382
FCD: 110850661
DMK: 116916001
DPZ: 124328406
PVM: 113821710
PJA: 122254312
PCHN: 125038629
PMOO: 119579061
HAME: 121858830
PCLA: 123746108
CQD: 128702290
PTRU: 123503477
MNZ: 135212033
EAF: 111696783
LSM: 121116178
TCF: 131887353
PPOI: 119094036
VDE: 111248336
VJA: 111268405
TUT: 107368315
DPTE: 113794273
DFR: 124498727
CSCU: 111620730
CVS: 136982187
PTEP: 107447707
ABRU: 129975780
SDM: 118182299
PMEO: 129593282
CEL: CELE_ZK1307.5(sqv-8)
CBR: CBG_01067(Cbr-sqv-8)
BMY: BM_BM7663(Bma-sqv-8)
PVUL: 126831843
GAE: 121371236
CRG: 105326288
CANU: 128175311
CVN: 111129954
OED: 125648932
MYI: 110466531
PMAX: 117323607
RPHI: 132722416
DPOL: 127874690
OBI: 106873166
OSN: 115217391
LJP: 135466529
LAK: 106166698
EGL: EGR_08995
HMG: 100200928
 » show all
Reference
  Authors
Ouzzine M, Gulberti S, Netter P, Magdalou J, Fournel-Gigleux S
  Title
Structure/function of the human Ga1beta1,3-glucuronosyltransferase. Dimerization and functional activity are mediated by two crucial cysteine residues.
  Journal
J Biol Chem 275:28254-60 (2000)
DOI:10.1074/jbc.M002182200
  Sequence
[hsa:26229]
Reference
  Authors
Kim BT, Tsuchida K, Lincecum J, Kitagawa H, Bernfield M, Sugahara K
  Title
Identification and characterization of three Drosophila melanogaster glucuronyltransferases responsible for the synthesis of the conserved glycosaminoglycan-protein linkage region of proteoglycans. Two novel homologs exhibit broad specificity toward oligosaccharides from proteoglycans, glycoproteins, and glycosphingolipids.
  Journal
J Biol Chem 278:9116-24 (2003)
DOI:10.1074/jbc.M209344200
  Sequence

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