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ataxin-1 (human)

Overview ataxin-1 spinocerebellar ataxia type 1 (SCA1) protein. Expansion of CAG repeats in the SCA1 gene results in an abnormally long polyglutamine tract in the protein, causing polyglutamine-induced neurodegeneration. 14-3-3 and Akt kinase stabilize both wild-type and mutant forms of ataxin-1. The expansion of the polyglutamine tract may alter this function. Note: This description may include information from UniProtKB. Protein type: RNA binding protein Cellular Component: nucleoplasm; nuclear RNA export factor complex; nuclear matrix; intracellular membrane-bound organelle; cytoplasm; nucleolus; nucleus; nuclear inclusion body Molecular Function: protein C-terminus binding; identical protein binding; protein binding; protein self-association; DNA binding; poly(U) binding; poly(rG) binding Biological Process: RNA processing; cell death; transcription, DNA-dependent; nuclear export; negative regulation of transcription, DNA-dependent Reference #:  P54253 (UniProtKB) Alt. Names/Synonyms: ataxin 1; Ataxin-1; ATX1; ATXN1; D6S504E; SCA1; Spinocerebellar ataxia type 1 protein Gene Symbols: ATXN1 Molecular weight: 86,923 Da Basal Isoelectric point: 8.49  Predict pI for various phosphorylation states CST Pathways:  PI3K/Akt Signaling Protein-Specific Antibodies or siRNAs from Cell Signaling Technology®

Select Structure to View Below ataxin-1 1OA8 - A/B/C/D=563-694 (human) 4APT - A/D/C/B=566-688 (human) 4AQP - A/D/C/B=566-688 (human) 4J2J - A/C/B=562-688 (human) 4J2L - A/B=562-688 (human)

STRING  |  Scansite  |  Phospho.ELM  |  NetworKIN  |  Pfam  |  RCSB PDB 1OA8 4APT 4AQP 4J2J 4J2L  |  Phospho3D  |  DISEASE 164400 601556  |  Source  |  GeneCards  |  UniProtKB  |  Entrez-Gene  |  Ensembl Gene

	Sites Implicated In
cytoskeletal reorganization: S775‑p intracellular localization: S775‑p molecular association, regulation: S775‑p protein degradation: S775‑p protein stabilization: S775‑p

	Modification Sites and Domains	Show Modification Legend
Click here to view phosphorylation modifications only

	Modification Sites in Parent Protein, Orthologs, and Isoforms	Show Modification Legend

SS  SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.	MS  MS: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.			human
1	0		K16-s	NECLPPKkREIPATS
0	1		P62	HGGGRHGPAGTSVEL
0	2		S82	IGLHKALSTGLDYsP
0	1		S82	IGLHKALSTGLDYsP
0	5		S88-p	LSTGLDYsPPSAPRS
1	0		K194-s	LSQTPGHkAEQQQQQ
1	1		T235-p	SRAPGLItPGsPPPA
1	7		S238-p	PGLItPGsPPPAQQN
0	2		A242	tPGsPPPAQQNQYVH
0	1		S252	NQYVHISSSPQNTGR
0	1		S253	QYVHISSSPQNTGRT
0	1		T257	ISSSPQNTGRTASPP
0	1		S312-p	ATKKAESsRLQQAIQ
0	3		S406-p	QATHREAsPSTLNDK
1	0		K609-s	AEISNDLkIDSSTVE
1	0		K696-s	NLKNGSVkKGQPVDP
0	1		K709	DPASVLLKHsKADGL
0	2		S711-p	ASVLLKHsKADGLAG
1	0		K745-s	LSENGELkFPEKMGL
10	14		S775-p	ATRKRRWsAPESRKL

	mouse
K16	NECLPPKKREIPATS
S62-p	HGGGRHGsAGTSGEH
S81-p	MGLHKALsAGLDYsP
S81-g	MGLHKALsAGLDYsP
S87-p	LsAGLDYsPPSAPRS
K193	LSQAPGHKVEPPPQQ
N210	SRAAGLVNPGsPPPP
S213-p	AGLVNPGsPPPPtQQ
T218-p	PGsPPPPtQQNQYIH
S228-p	NQYIHISssPQSsGR
S229-p	QYIHISssPQSsGRA
S233-p	ISssPQSsGRATSPP
S288	STKKAESSRLQQAMQ
S382-p	QTTHREAsPSTLNDK
K585	AEISNDLKIDSSTVE
K672	NLKNGSVKKGQPVDP
K685-u	DPASVLLkQAKTDSL
A687	ASVLLkQAKTDSLAG
K721	LSENGELKFPEKIGL
S751-p	ATRKRRWsAPETRKL

	rat
K16	NECLPPKKREIPATS
P62	HGGGRHGPAGTSGEH
S81	MGLHKALSAGLDYSP
S81	MGLHKALSAGLDYSP
S87	LSAGLDYSPPSAPRS
K193	LSQAPGHKVEPPPQQ
N210	GRAAGLVNPGSPPPt
S213	AGLVNPGSPPPtQQN
T217-p	NPGSPPPtQQNQYIH
S227	NQYIHISSSPQSSGR
S228	QYIHISSSPQSSGRA
S232	ISSSPQSSGRATSPP
S286	STKKAESSRLQQAMQ
S380-p	QATHREAsPSTLNDK
K583	AEISNDLKIDSSTVE
K670	NLKNGSVKKGQPVDP
K683	DPASALLKHAKTDSL
A685	ASALLKHAKTDSLAG
K719	LSENGELKFPEKIGL
S749	ATRKRRWSAPETRKL

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