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What is Reactome ?
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Our Logo
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Privacy Notice
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Table of Contents
DOIs
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ATXN3 [cytosol]
Stable Identifier
R-HSA-5625607
Type
Protein [EntityWithAccessionedSequence]
Species
Homo sapiens
Compartment
cytosol
Synonyms
Ataxin-3, ATX3_HUMAN
Locations in the PathwayBrowser for interactor UniProt:Q9H8Y8
Expand all
Metabolism of proteins (Homo sapiens)
Post-translational protein modification (Homo sapiens)
Deubiquitination (Homo sapiens)
Josephin domain DUBs (Homo sapiens)
ATXN3 binds polyUb-PARK2 (Homo sapiens)
ATXN3 [cytosol] (Homo sapiens)
External Reference Information
External Reference
UniProt:P54252 ATXN3
Gene Names
ATXN3, ATX3, MJD, MJD1, SCA3
Chain
chain:1-361
Reference Genes
BioGPS Gene:4287 ATXN3
COSMIC (genes):ATXN3 ATXN3
CTD Gene:4287 ATXN3
dbSNP Gene:4287 ATXN3
ENSEMBL:ENSG00000066427 ATXN3
HGNC:7106 ATXN3
KEGG Gene (Homo sapiens):4287 ATXN3
Monarch:4287 ATXN3
NCBI Gene:4287 ATXN3
OMIM:607047 ATXN3
UCSC:P54252 ATXN3
Reference Transcript
RefSeq:NM_001127696.1 ATXN3
RefSeq:NM_004993.5 ATXN3
RefSeq:NM_001164780.1 ATXN3
RefSeq:NM_030660.4 ATXN3
Other Identifiers
0002470605
0004150735
11726361_a_at
11726362_a_at
11726363_x_at
16796020
205415_s_at
205416_s_at
235240_at
238723_at
3576888
3576890
3576891
3576892
3576893
3576894
3576895
3576896
3576897
3576898
3576899
3576903
3576904
3576909
3576910
3576911
3576912
3576914
3576915
3576920
3576923
3576926
3576928
3576929
3576933
36819_at
4287
56787_at
7980940
82526_at
A_23_P129031
A_24_P13595
A_33_P3275310
A_33_P3320762
GE59310
GO:0000226
GO:0003674
GO:0004843
GO:0005515
GO:0005575
GO:0005622
GO:0005634
GO:0005654
GO:0005730
GO:0005737
GO:0005739
GO:0005759
GO:0005789
GO:0005829
GO:0005886
GO:0006259
GO:0006289
GO:0006464
GO:0006508
GO:0006511
GO:0006515
GO:0006950
GO:0007010
GO:0007155
GO:0007267
GO:0007268
GO:0007399
GO:0008150
GO:0008233
GO:0008234
GO:0009056
GO:0010810
GO:0016363
GO:0016579
GO:0016787
GO:0019899
GO:0030036
GO:0031625
GO:0031966
GO:0034605
GO:0034641
GO:0035520
GO:0036459
GO:0042405
GO:0043161
GO:0043226
GO:0045104
GO:0045202
GO:0048856
GO:0051117
GO:0061578
GO:0070536
GO:0071108
GO:0071218
GO:1904294
GO:1904379
GO:1990380
Hs.142043.0.A1_3p_at
Hs.46648.0.A1_3p_at
Hs.66521.0.S1_3p_a_at
ILMN_1725696
ILMN_2393497
PH_hs_0025115
TC14002125.hg
g13518018_3p_a_at
Participant Of
hasMember
ATXN3,ATXN3L,JOSD1,JOSD2 [cytosol]
input
ATXN3 binds polyUb-PARK2
hasComponent
ATXN3:PARK2 [cytosol]
ATXN3:polyUb-PARK2 [cytosol]
Other forms of this molecule
ATXN3 [nucleoplasm]
Inferred To
ATXN3 [cytosol] (Bos taurus)
ATXN3 [cytosol] (Gallus gallus)
ATXN3 [cytosol] (Plasmodium falciparum)
ATXN3 [cytosol] (Sus scrofa)
Atxn3 [cytosol] (Mus musculus)
Atxn3 [cytosol] (Rattus norvegicus)
osbpl3 [cytosol] (Xenopus tropicalis)
TRIP11 [cytosol] (Canis familiaris)
atx-3 [cytosol] (Caenorhabditis elegans)
atxn3 [cytosol] (Danio rerio)
Cross References
RefSeq
NP_001121168.1
,
NP_004984.2
,
NP_001158252.1
,
NP_109376.1
OpenTargets
ENSG00000066427
HPA
ENSG00000066427-ATXN3
GeneCards
P54252
Ensembl
ENST00000502250
,
ENSG00000066427
,
ENSP00000437157
,
ENST00000644486
,
ENSP00000425322
,
ENST00000503767
,
ENSP00000339110
,
ENSP00000426697
,
ENSP00000496695
,
ENST00000340660
,
ENST00000532032
PRO
P54252
Orphanet
15339
PDB
4YS9
,
4WTH
,
2DOS
,
2KLZ
,
1YZB
,
2JRI
,
2AGA
Interactors (105)
Accession
#Entities
Entities
Confidence Score
Evidence (IntAct)
UniProt:P55072 VCP
8
VCP [endoplasmic reticulum membrane]
(R-HSA-8943105)
Me3K315-VCP [cytosol]
(R-HSA-8932209)
VCP [nucleoplasm]
(R-HSA-5643745)
VCP [azurophil granule lumen]
(R-HSA-6800438)
VCP [ficolin-1-rich granule lumen]
(R-HSA-6800425)
VCP [extracellular region]
(R-HSA-6806191)
VCP [secretory granule lumen]
(R-HSA-6801039)
VCP [cytosol]
(R-HSA-1181251)
0.675
4
UniProt:Q01844 EWS
0.666
4
UniProt:Q9BSU1 CP070
0.666
4
UniProt:Q14CB8 ARHGAP19
1
ARHGAP19 [cytosol]
(R-HSA-200767)
0.666
4
UniProt:P54725 RAD23A
1
RAD23A [nucleoplasm]
(R-HSA-5688076)
0.666
9
UniProt:Q8IUI8 CRLF3
0.556
3
UniProt:Q9UHY8 FEZ2
0.556
6
UniProt:P50570-2 DNM2
0.556
3
UniProt:Q8TBK5 Q8TBK5
0.556
9
UniProt:O75330-3 HMMR
0.556
3
UniProt:Q8TB36 GDAP1
2
GDAP1 [cytosol]
(R-HSA-9603796)
GDAP1 [peroxisomal membrane]
(R-HSA-9603788)
0.556
3
UniProt:Q8N895 ZN366
0.556
3
UniProt:P29466-3 CASP1
0.556
9
UniProt:Q9P1A6-3 DLGAP2
0.556
3
UniProt:Q96DC9-2 Q96DC92
0.556
9
UniProt:Q3B7T1 EDRF1
0.556
3
UniProt:Q9H4I2-2 ZHX3
0.556
3
UniProt:Q9Y483-4 MTF2
0.556
3
UniProt:Q8IYF3-3 TEX11
0.556
6
UniProt:Q9H8Y8 GORASP2
2
p-T222,T225-GORASP2 [Golgi membrane]
(R-HSA-2422965)
GORASP2 [Golgi membrane]
(R-HSA-2422385)
0.556
3
UniProt:Q9H8H3 METTL7A
2
METTL7A [tertiary granule lumen]
(R-HSA-6800170)
METTL7A [extracellular region]
(R-HSA-6800468)
0.556
3
UniProt:Q6UY14-3 ADAMTSL4
0.556
3
UniProt:Q9Y458 TBX22
0.556
3
UniProt:Q9HBE1-4 PATZ1
0.556
3
UniProt:Q96EP0 RNF31
1
RNF31 [cytosol]
(R-HSA-5212660)
0.556
3
UniProt:Q96G97-4 BSCL2
0.556
3
UniProt:Q6PCB6 ABHD17C
1
ABHD17C [plasma membrane]
(R-HSA-9647774)
0.556
3
UniProt:Q9H7C9 AAMDC
0.556
3
UniProt:Q8N5Z7 Q8N5Z7
0.556
6
UniProt:Q9NS23-4 RASSF1
0.556
3
UniProt:Q6ZNA4-2 Q6ZNA42
0.556
6
UniProt:Q8NEZ2 VPS37A
1
VPS37A [endosome membrane]
(R-HSA-184333)
0.556
3
UniProt:Q9NQ40 SLC52A3
1
SLC52A3 [plasma membrane]
(R-HSA-3165247)
0.556
3
UniProt:Q8NBT0 POC1A
0.556
3
UniProt:Q8TDW5-2 SYTL5
0.556
3
UniProt:Q16740 CLPP
0.556
3
UniProt:P37173 TGFBR2
9
TGFBR2 E526Q [plasma membrane]
(R-HSA-3645770)
TGFBR2 Y470D [plasma membrane]
(R-HSA-3640905)
TGFBR2 D522N [plasma membrane]
(R-HSA-3640907)
TGFBR2 R528H [plasma membrane]
(R-HSA-3640894)
TGFBR2 R528C [plasma membrane]
(R-HSA-3640899)
TGFBR2 K128fs*3 [cytosol]
(R-HSA-3642173)
TGFBR2 K128fs*35 [cytosol]
(R-HSA-3642166)
NEDD8-K556,K567-TGFBR2 [plasma membrane]
(R-HSA-4332222)
TGFBR2 [plasma membrane]
(R-HSA-170842)
0.556
3
UniProt:P40227 CCT6A
1
CCT6A [cytosol]
(R-HSA-390454)
0.556
3
UniProt:Q96DC9 OTUB2
2
OTUB2 [cytosol]
(R-HSA-933410)
OTUB2 [nucleoplasm]
(R-HSA-6783260)
0.556
9
UniProt:Q8WY64 MYLIP
1
MYLIP [cytosol]
(R-HSA-8850908)
0.556
3
UniProt:Q92995 USP13
2
USP13 [cytosol]
(R-HSA-6781893)
USP13 [nucleoplasm]
(R-HSA-6781924)
0.556
3
UniProt:O95551 TDP2
1
TDP2 [nucleoplasm]
(R-HSA-5687253)
0.556
3
UniProt:Q9NRP7 STK36
0.556
3
UniProt:O43488 AKR7A2
1
AKR7A2 [cytosol]
(R-HSA-5423681)
0.556
3
UniProt:P06241-3 FYN
0.556
3
UniProt:Q99932-2 SPAG8
0.556
6
UniProt:P42336 PIK3CA
25
PIK3CA H1047R [cytosol]
(R-HSA-1500716)
PIK3CA H1047L [cytosol]
(R-HSA-1500714)
PIK3CA M1043I [cytosol]
(R-HSA-1500719)
PIK3CA M1043T [cytosol]
(R-HSA-2395934)
PIK3CA M1043V [cytosol]
(R-HSA-2395932)
PIK3CA H1047Y [cytosol]
(R-HSA-1500720)
PIK3CA E542K [cytosol]
(R-HSA-1500701)
PIK3CA Q546K [cytosol]
(R-HSA-1500705)
PIK3CA E545A [cytosol]
(R-HSA-2362355)
PIK3CA E545K [cytosol]
(R-HSA-1500706)
PIK3CA E545Q [cytosol]
(R-HSA-2362362)
PIK3CA Q546E [cytosol]
(R-HSA-2362383)
PIK3CA E545V [cytosol]
(R-HSA-2362365)
PIK3CA E542V [cytosol]
(R-HSA-2328074)
PIK3CA Q546L [cytosol]
(R-HSA-2362375)
PIK3CA E545G [cytosol]
(R-HSA-1500709)
PIK3CA Q546H [cytosol]
(R-HSA-2362374)
PIK3CA Q546R [cytosol]
(R-HSA-2362373)
PIK3CA Q546P [cytosol]
(R-HSA-1500695)
PIK3CA E542Q [cytosol]
(R-HSA-2328003)
PIK3CA R38H [cytosol]
(R-HSA-1500718)
PIK3CA R38S [cytosol]
(R-HSA-2395947)
PIK3CA R38G [cytosol]
(R-HSA-2395950)
PIK3CA R38C [cytosol]
(R-HSA-1500691)
PIK3CA [cytosol]
(R-HSA-74787)
0.556
3
UniProt:Q13064 MKRN3
0.556
3
UniProt:P42574 CASP3
5
CASP3(176-277) [nucleoplasm]
(R-HSA-211203)
CASP3(29-175) [nucleoplasm]
(R-HSA-211194)
CASP3(1-277) [cytosol]
(R-HSA-57021)
CASP3(29-175) [cytosol]
(R-HSA-141644)
CASP3(176-277) [cytosol]
(R-HSA-141646)
0.556
9
UniProt:O94851 MICA2
0.556
3
UniProt:Q9Y2M5 KLHL20
1
KLHL20 [cytosol]
(R-HSA-976032)
0.556
3
UniProt:A4FUJ8 A4FUJ8
0.556
3
UniProt:Q8NA77 TEX19
0.556
3
IntAct:EBI-25958427 CCSB
0.556
9
UniProt:Q3SYB3 FX4L6
0.556
3
IntAct:EBI-25974350 CCSB
0.556
3
UniProt:Q15554-4 Q155544
0.556
3
IntAct:EBI-25866689 CCSB
0.556
3
UniProt:Q9UDY8-2 MALT1
0.556
3
UniProt:Q8IVP1 Q8IVP1
0.556
3
UniProt:Q9UMX0 UBQLN1
1
UBQLN1 [cytosol]
(R-HSA-8866006)
0.556
6
UniProt:Q02930-3 CREB5
0.556
3
UniProt:Q01844-3 EWSR1
0.556
9
UniProt:P68431 H3C1
59
2xAcK-HIST1H3A [nucleoplasm]
(R-HSA-427507)
HIST1H3A [extracellular region]
(R-HSA-977570)
Ack-Me3K5-HIST1H3A [nucleoplasm]
(R-HSA-4551293)
AcK-Me3K10-HIST1H3A [nucleoplasm]
(R-HSA-4551291)
AcK-Me3K28-HIST1H3A [nucleoplasm]
(R-HSA-4551292)
AcK19-HIST1H3A [nucleoplasm]
(R-HSA-4568761)
AcK5-HIST1H3A [nucleoplasm]
(R-HSA-4568751)
AcK24-HIST1H3A [nucleoplasm]
(R-HSA-4568754)
AcK15-HIST1H3A [nucleoplasm]
(R-HSA-4568743)
Ack15-HIST1H3A [nucleoplasm]
(R-HSA-4549217)
Me2aR27-HIST1H3A [nucleoplasm]
(R-HSA-5229052)
MeR27-HIST1H3A [nucleoplasm]
(R-HSA-5229050)
Me2aR18-HIST1H3A [nucleoplasm]
(R-HSA-5229062)
Me2sR9-HIST1H3A [nucleoplasm]
(R-HSA-5211328)
Me2sR3-HIST1H3A [nucleoplasm]
(R-HSA-5218935)
AcK9,14-p-S10-HIST1H3A [nucleoplasm]
(R-HSA-8870893)
AcK14,18,79-p-S10-HIST1H3A [nucleoplasm]
(R-HSA-8871271)
AcK9,14,18-p-S10,T11-HIST1H3A [nucleoplasm]
(R-HSA-8871306)
AcK14,18-p-S10,T11-HIST1H3A [nucleoplasm]
(R-HSA-8871294)
AcK9,14,18,79-p-S10,T11-HIST1H3A [nucleoplasm]
(R-HSA-8865596)
AcK14,18-p-S10-HIST1H3A [nucleoplasm]
(R-HSA-8871295)
AcK14,18,79-p-T11-HIST1H3A [nucleoplasm]
(R-HSA-8871288)
AcK14,18-p-T11-HIST1H3A [nucleoplasm]
(R-HSA-8871299)
AcK14,18,79-p-S10,T11-HIST1H3A [nucleoplasm]
(R-HSA-8871286)
Me3K10-HIST1H3A [nucleoplasm]
(R-HSA-4754188)
Me3K28-HIST1H3A [nucleoplasm]
(R-HSA-4754169)
Me2K28-HIST1H3A [nucleoplasm]
(R-HSA-4754193)
MeK37-HIST1H3A [nucleoplasm]
(R-HSA-4722135)
MeK80-HIST1H3A [nucleoplasm]
(R-HSA-5649791)
Me3K80-HIST1H3A [nucleoplasm]
(R-HSA-5649795)
Me2K80-HIST1H3A [nucleoplasm]
(R-HSA-5649768)
Me3K37-HIST1H3A [nucleoplasm]
(R-HSA-5638160)
Me2K37-HIST1H3A [nucleoplasm]
(R-HSA-4722125)
MeK10-HIST1H3A [nucleoplasm]
(R-HSA-212105)
MeK28-HIST1H3A [nucleoplasm]
(R-HSA-5638327)
MeK-10-HIST1H3A [cytosol]
(R-HSA-8943830)
HIST1H3A [cytosol]
(R-HSA-8943829)
Me2aR3-HIST1H3A [nucleoplasm]
(R-HSA-5244526)
MeK5-HIST1H3A [nucleoplasm]
(R-HSA-4687016)
Cit27-HIST1H3A [nucleoplasm]
(R-HSA-5246532)
Cit3-HIST1H3A [nucleoplasm]
(R-HSA-5246544)
Cit9-HIST1H3A [nucleoplasm]
(R-HSA-5246530)
Cit18-HIST1H3A [nucleoplasm]
(R-HSA-5246534)
MeR18-HIST1H3A [nucleoplasm]
(R-HSA-5229059)
MeR3-HIST1H3A [nucleoplasm]
(R-HSA-5244529)
MeR27-HIST1H3A [nucleoplasm]
(R-HSA-5244801)
MeR9-HIST1H3A [nucleoplasm]
(R-HSA-5244806)
Ack10-HIST1H3A [nucleoplasm]
(R-HSA-4568939)
Me2K10-HIST1H3A [nucleoplasm]
(R-HSA-427378)
Me2K5,Me2aR3-HIST1H3A [nucleoplasm]
(R-HSA-8936572)
p-T12, Me2K-10-HIST1H3A [nucleoplasm]
(R-HSA-5625790)
p-T12-HIST1H3A [nucleoplasm]
(R-HSA-5625872)
p-T12,MeK-10-HIST1H3A [nucleoplasm]
(R-HSA-5625859)
p-T12, Me3K-10-HIST1H3A [nucleoplasm]
(R-HSA-5625757)
Me3K-10-HIST1H3A [nucleoplasm]
(R-HSA-427734)
Me3K5-HIST1H3A [nucleoplasm]
(R-HSA-1214186)
HIST1H3A [nucleoplasm]
(R-HSA-212070)
Me3K-28-HIST1H3A [nucleoplasm]
(R-HSA-212220)
Me2K5-HIST1H3A [nucleoplasm]
(R-HSA-1214170)
0.556
3
UniProt:Q6UWD8 CP054
0.556
3
UniProt:Q9Y4R8 TELO2
0.556
3
UniProt:Q01844-4 EWSR1
0.556
3
UniProt:Q96FT7-4 ASIC4
0.556
3
UniProt:O95416 SOX14
0.556
3
UniProt:Q9UPM6 LHX6
0.556
3
UniProt:Q8IUW3 SPA2L
0.556
3
UniProt:Q66K80 RUAS1
0.556
3
UniProt:Q8N865 CG031
0.556
3
UniProt:Q96LX7-5 CCDC17
0.556
3
UniProt:O15530-4 PDPK1
0.556
3
IntAct:EBI-25973200 CCSB
0.556
3
UniProt:Q9NP61 ARFGAP3
1
ARFGAP3 [cytosol]
(R-HSA-6807740)
0.556
3
UniProt:P51665 PSMD7
5
PSMD7 [extracellular region]
(R-HSA-6806488)
PSMD7 [ficolin-1-rich granule lumen]
(R-HSA-6800406)
PSMD7 [secretory granule lumen]
(R-HSA-6801008)
PSMD7 [nucleoplasm]
(R-HSA-174309)
PSMD7 [cytosol]
(R-HSA-68806)
0.556
9
IntAct:EBI-25831191 CCSB
0.556
3
UniProt:Q9Y575-3 ASB3
0.556
3
UniProt:Q8N3R9 MPP5
1
MPP5 [plasma membrane]
(R-HSA-419969)
0.556
3
UniProt:Q03405-2 PLAUR
0.556
3
UniProt:Q15007-2 WTAP
0.556
3
UniProt:Q9BWI9 Q9BWI9
0.556
6
UniProt:P13051-2 UNG
0.556
3
UniProt:Q8NFH3 NUP43
2
NUP43 [cytosol]
(R-HSA-376243)
NUP43 [nuclear envelope]
(R-HSA-157739)
0.556
3
UniProt:Q5VV42 CDKAL1
1
CDKAL1 [endoplasmic reticulum membrane]
(R-HSA-6786612)
0.556
3
UniProt:P06276 BCHE
2
BCHE [plasma membrane]
(R-HSA-3640835)
BCHE [extracellular region]
(R-HSA-434469)
0.556
3
UniProt:P22692 IGFBP4
5
p-IGFBP4 [endoplasmic reticulum lumen]
(R-HSA-8957038)
IGFBP4 [endoplasmic reticulum lumen]
(R-HSA-8956754)
IGFBP4 [extracellular region]
(R-HSA-381421)
IGFBP4(157-237) [extracellular region]
(R-HSA-381510)
IGFBP4(22-156) [extracellular region]
(R-HSA-381516)
0.556
3
UniProt:Q9H816 DCLRE1B
1
DCLRE1B [nucleoplasm]
(R-HSA-6785735)
0.556
3
UniProt:O75409 HYPM
0.556
3
UniProt:Q15466 NR0B2
1
NR0B2 [nucleoplasm]
(R-HSA-376312)
0.556
3
UniProt:P23142-4 FBLN1
0.556
3
UniProt:Q96NC0 ZMAT2
0.556
3
UniProt:Q93062-3 RBPMS
0.556
3
UniProt:P16581 SELE
1
SELE [plasma membrane]
(R-HSA-2870223)
0.556
3
UniProt:Q969S8 HDAC10
1
HDAC10 [nucleoplasm]
(R-HSA-351579)
0.556
3
UniProt:O75925 PIAS1
1
PIAS1 [nucleoplasm]
(R-HSA-877307)
0.556
9
UniProt:Q9H0M0 WWP1
1
WWP1 [cytosol]
(R-HSA-976037)
0.556
3
UniProt:P17612 PRKACA
3
PRKACA [cytosol]
(R-HSA-57844)
PRKACA [ciliary base]
(R-HSA-5610402)
PRKACA [nucleoplasm]
(R-HSA-111936)
0.556
3
UniProt:Q9BXR5 TLR10
1
TLR10 [plasma membrane]
(R-HSA-167926)
0.556
3
UniProt:O15287 FANCG
1
FANCG [nucleoplasm]
(R-HSA-419532)
0.556
3
UniProt:P50454 SERPINH1
1
SERPINH1 [endoplasmic reticulum lumen]
(R-HSA-2127480)
0.556
3
UniProt:Q9BYZ2 LDHAL6B
1
LDHAL6B [mitochondrial matrix]
(R-HSA-6807613)
0.556
3
UniProt:Q9NVD7 PARVA
1
PARVA [cytosol]
(R-HSA-432848)
0.556
9
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