CPLM 1.0 - Compendium of Protein Lysine Modification
TagContent
CPLM ID CPLM-008843
UniProt Accession
Genbank Protein ID
Genbank Nucleotide ID
Protein Name
 Ataxin-3 
Protein Synonyms/Alias
 Machado-Joseph disease protein 1; Spinocerebellar ataxia type 3 protein 
Gene Name
 ATXN3 
Gene Synonyms/Alias
 ATX3; MJD; MJD1; SCA3 
Created Date
 July 27, 2013 
Organism
 Homo sapiens (Human) 
NCBI Taxa ID
 9606 
Lysine Modification
Position
Peptide
Type
References
190VQQMHRPKLIGEELAubiquitination[1]
200GEELAQLKEQRVHKTubiquitination[1]
206LKEQRVHKTDLERVLubiquitination[1]
291RREAYFEKQQQKQQQubiquitination[1]
Reference
 [1] Landscape of the PARKIN-dependent ubiquitylome in response to mitochondrial depolarization.
 Sarraf SA, Raman M, Guarani-Pereira V, Sowa ME, Huttlin EL, Gygi SP, Harper JW.
 Nature. 2013 Apr 18;496(7445):372-6. [PMID: 23503661] 
Functional Description
 Deubiquitinating enzyme involved in protein homeostasis maintenance, transcription, cytoskeleton regulation, myogenesis and degradation of misfolded chaperone substrates. Binds long polyubiquitin chains and trims them, while it has weak or no activity against chains of 4 or less ubiquitins. Involved in degradation of misfolded chaperone substrates via its interaction with STUB1/CHIP: recruited to monoubiquitinated STUB1/CHIP, and restricts the length of ubiquitin chain attached to STUB1/CHIP substrates and preventing further chain extension. In response to misfolded substrate ubiquitination, mediates deubiquitination of monoubiquitinated STUB1/CHIP. Interacts with key regulators of transcription and represses transcription: acts as a histone- binding protein that regulates transcription. 
Sequence Annotation
 DOMAIN 1 180 Josephin.
 REPEAT 224 243 UIM 1.
 REPEAT 244 263 UIM 2.
 REPEAT 331 348 UIM 3.
 ACT_SITE 14 14 Nucleophile.
 ACT_SITE 119 119 Proton acceptor (Probable).
 ACT_SITE 134 134 Probable.
 MOD_RES 219 219 Phosphoserine (By similarity).  
Keyword
 3D-structure; Alternative splicing; Complete proteome; Hydrolase; Neurodegeneration; Nucleus; Phosphoprotein; Polymorphism; Protease; Reference proteome; Repeat; Spinocerebellar ataxia; Thiol protease; Transcription; Transcription regulation; Triplet repeat expansion; Ubl conjugation; Ubl conjugation pathway. 
Sequence Source
 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 
Protein Length
 364 AA 
Protein Sequence
MESIFHEKQE GSLCAQHCLN NLLQGEYFSP VELSSIAHQL DEEERMRMAE GGVTSEDYRT 60
FLQQPSGNMD DSGFFSIQVI SNALKVWGLE LILFNSPEYQ RLRIDPINER SFICNYKEHW 120
FTVRKLGKQW FNLNSLLTGP ELISDTYLAL FLAQLQQEGY SIFVVKGDLP DCEADQLLQM 180
IRVQQMHRPK LIGEELAQLK EQRVHKTDLE RVLEANDGSG MLDEDEEDLQ RALALSRQEI 240
DMEDEEADLR RAIQLSMQGS SRNISQDMTQ TSGTNLTSEE LRKRREAYFE KQQQKQQQQQ 300
QQQQQGDLSG QSSHPCERPA TSSGALGSDL GDAMSEEDML QAAVTMSLET VRNDLKTEGK 360
K 361 
Gene Ontology
 GO:0005737; C:cytoplasm; TAS:ProtInc.
 GO:0005759; C:mitochondrial matrix; IEA:Compara.
 GO:0031966; C:mitochondrial membrane; IEA:Compara.
 GO:0042405; C:nuclear inclusion body; IEA:Compara.
 GO:0016363; C:nuclear matrix; IEA:UniProtKB-SubCell.
 GO:0005654; C:nucleoplasm; TAS:ProtInc.
 GO:0008242; F:omega peptidase activity; IEA:InterPro.
 GO:0001012; F:RNA polymerase II regulatory region DNA binding; IEA:Compara.
 GO:0031625; F:ubiquitin protein ligase binding; ISS:UniProtKB.
 GO:0004221; F:ubiquitin thiolesterase activity; ISS:UniProtKB.
 GO:0004843; F:ubiquitin-specific protease activity; ISS:UniProtKB.
 GO:0030036; P:actin cytoskeleton organization; IMP:MGI.
 GO:0008219; P:cell death; IEA:UniProtKB-KW.
 GO:0034605; P:cellular response to heat; IEA:Compara.
 GO:0071218; P:cellular response to misfolded protein; ISS:UniProtKB.
 GO:0035640; P:exploration behavior; IEA:Compara.
 GO:0070932; P:histone H3 deacetylation; IEA:Compara.
 GO:0045104; P:intermediate filament cytoskeleton organization; IMP:MGI.
 GO:0000226; P:microtubule cytoskeleton organization; IMP:MGI.
 GO:0006515; P:misfolded or incompletely synthesized protein catabolic process; ISS:UniProtKB.
 GO:0035520; P:monoubiquitinated protein deubiquitination; ISS:UniProtKB.
 GO:0007399; P:nervous system development; TAS:ProtInc.
 GO:0006289; P:nucleotide-excision repair; TAS:ProtInc.
 GO:0043161; P:proteasomal ubiquitin-dependent protein catabolic process; ISS:UniProtKB.
 GO:0010810; P:regulation of cell-substrate adhesion; IMP:MGI.
 GO:0006355; P:regulation of transcription, DNA-dependent; IEA:UniProtKB-KW.
 GO:0007268; P:synaptic transmission; TAS:ProtInc.
 GO:0006351; P:transcription, DNA-dependent; IEA:UniProtKB-KW. 
Interpro
 IPR006155; Josephin.
 IPR003903; Ubiquitin-int_motif. 
Pfam
 PF02099; Josephin
 PF02809; UIM 
SMART
 SM00726; UIM 
PROSITE
 PS50957; JOSEPHIN
 PS50330; UIM 
PRINTS
 PR01233; JOSEPHIN.