CPLM 1.0 - Compendium of Protein Lysine Modification
TagContent
CPLM ID CPLM-008844
UniProt Accession
Genbank Protein ID
Genbank Nucleotide ID
Protein Name
 Ataxin-1 
Protein Synonyms/Alias
 Spinocerebellar ataxia type 1 protein 
Gene Name
 ATXN1 
Gene Synonyms/Alias
 ATX1; SCA1 
Created Date
 July 27, 2013 
Organism
 Homo sapiens (Human) 
NCBI Taxa ID
 9606 
Lysine Modification
Position
Peptide
Type
References
16NECLPPKKREIPATSsumoylation[1]
194LSQTPGHKAEQQQQQsumoylation[1]
609AEISNDLKIDSSTVEsumoylation[1]
696NLKNGSVKKGQPVDPsumoylation[1]
697LKNGSVKKGQPVDPAsumoylation[1]
745LSENGELKFPEKMGLsumoylation[1]
Reference
 [1] SUMOylation of the polyglutamine repeat protein, ataxin-1, is dependent on a functional nuclear localization signal.
 Riley BE, Zoghbi HY, Orr HT.
 J Biol Chem. 2005 Jun 10;280(23):21942-8. [PMID: 15824120] 
Functional Description
 Chromatin-binding factor that repress Notch signaling in the absence of Notch intracellular domain by acting as a CBF1 corepressor. Binds to the HEY promoter and might assist, along with NCOR2, RBPJ-mediated repression. Binds RNA in vitro. May be involved in RNA metabolism. The expansion of the polyglutamine tract may alter this function. 
Sequence Annotation
 DOMAIN 562 693 AXH.
 REGION 494 604 Self-association.
 REGION 538 815 Interaction with USP7.
 REGION 540 766 RNA-binding.
 MOTIF 794 797 Nuclear localization signal (By
 MOD_RES 238 238 Phosphoserine.
 MOD_RES 775 775 Phosphoserine.
 CROSSLNK 16 16 Glycyl lysine isopeptide (Lys-Gly)
 CROSSLNK 194 194 Glycyl lysine isopeptide (Lys-Gly)
 CROSSLNK 609 609 Glycyl lysine isopeptide (Lys-Gly)
 CROSSLNK 696 696 Glycyl lysine isopeptide (Lys-Gly)
 CROSSLNK 745 745 Glycyl lysine isopeptide (Lys-Gly)  
Keyword
 3D-structure; Alternative splicing; Complete proteome; Cytoplasm; DNA-binding; Isopeptide bond; Neurodegeneration; Nucleus; Phosphoprotein; Polymorphism; Reference proteome; Repressor; RNA-binding; Spinocerebellar ataxia; Transcription; Transcription regulation; Triplet repeat expansion; Ubl conjugation. 
Sequence Source
 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 
Protein Length
 815 AA 
Protein Sequence
MKSNQERSNE CLPPKKREIP ATSRSSEEKA PTLPSDNHRV EGTAWLPGNP GGRGHGGGRH 60
GPAGTSVELG LQQGIGLHKA LSTGLDYSPP SAPRSVPVAT TLPAAYATPQ PGTPVSPVQY 120
AHLPHTFQFI GSSQYSGTYA SFIPSQLIPP TANPVTSAVA SAAGATTPSQ RSQLEAYSTL 180
LANMGSLSQT PGHKAEQQQQ QQQQQQQQHQ HQQQQQQQQQ QQQQQHLSRA PGLITPGSPP 240
PAQQNQYVHI SSSPQNTGRT ASPPAIPVHL HPHQTMIPHT LTLGPPSQVV MQYADSGSHF 300
VPREATKKAE SSRLQQAIQA KEVLNGEMEK SRRYGAPSSA DLGLGKAGGK SVPHPYESRH 360
VVVHPSPSDY SSRDPSGVRA SVMVLPNSNT PAADLEVQQA THREASPSTL NDKSGLHLGK 420
PGHRSYALSP HTVIQTTHSA SEPLPVGLPA TAFYAGTQPP VIGYLSGQQQ AITYAGSLPQ 480
HLVIPGTQPL LIPVGSTDME ASGAAPAIVT SSPQFAAVPH TFVTTALPKS ENFNPEALVT 540
QAAYPAMVQA QIHLPVVQSV ASPAAAPPTL PPYFMKGSII QLANGELKKV EDLKTEDFIQ 600
SAEISNDLKI DSSTVERIED SHSPGVAVIQ FAVGEHRAQV SVEVLVEYPF FVFGQGWSSC 660
CPERTSQLFD LPCSKLSVGD VCISLTLKNL KNGSVKKGQP VDPASVLLKH SKADGLAGSR 720
HRYAEQENGI NQGSAQMLSE NGELKFPEKM GLPAAPFLTK IEPSKPAATR KRRWSAPESR 780
KLEKSEDEPP LTLPKPSLIP QEVKICIEGR SNVGK 815 
Gene Ontology
 GO:0005737; C:cytoplasm; IDA:UniProtKB.
 GO:0042405; C:nuclear inclusion body; IDA:UniProtKB.
 GO:0016363; C:nuclear matrix; IDA:UniProtKB.
 GO:0005654; C:nucleoplasm; IDA:UniProtKB.
 GO:0003677; F:DNA binding; IEA:UniProtKB-KW.
 GO:0034046; F:poly(G) RNA binding; IDA:UniProtKB.
 GO:0008266; F:poly(U) RNA binding; IDA:UniProtKB.
 GO:0043621; F:protein self-association; IDA:UniProtKB.
 GO:0008344; P:adult locomotory behavior; IEA:Compara.
 GO:0008219; P:cell death; IEA:UniProtKB-KW.
 GO:0043569; P:negative regulation of insulin-like growth factor receptor signaling pathway; IEA:Compara.
 GO:0042326; P:negative regulation of phosphorylation; IEA:Compara.
 GO:0045892; P:negative regulation of transcription, DNA-dependent; IDA:UniProtKB.
 GO:0051168; P:nuclear export; IDA:UniProtKB.
 GO:0045944; P:positive regulation of transcription from RNA polymerase II promoter; IEA:Compara.
 GO:0060079; P:regulation of excitatory postsynaptic membrane potential; IEA:Compara.
 GO:0006396; P:RNA processing; NAS:UniProtKB.
 GO:0006351; P:transcription, DNA-dependent; IEA:UniProtKB-KW.
 GO:0008542; P:visual learning; IEA:Compara. 
Interpro
 IPR013723; Ataxin-1_HBP1.
 IPR003652; Ataxin_AXH_dom.
 IPR020997; Capicua_tscrpt_rep_mod. 
Pfam
 PF12547; ATXN-1_C
 PF08517; AXH 
SMART
 SM00536; AXH 
PROSITE
 PS51148; AXH 
PRINTS