ATXN3
UniProt ID: P54252
Entrez ID: 4287
Protein name: Ataxin-3 (EC 3.4.19.12) (Machado-Joseph disease protein 1) (Spinocerebellar ataxia type 3 protein)
External links: UniprotKB HGNC RefSeq COSMIC
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FUNCTION: Deubiquitinating enzyme involved in protein homeostasis maintenance, transcription, cytoskeleton regulation, myogenesis and degradation of misfolded chaperone substrates (PubMed:12297501, PubMed:17696782, PubMed:23625928, PubMed:28445460, PubMed:16118278). Binds long polyubiquitin chains a...
FUNCTION: Deubiquitinating enzyme involved in protein homeostasis maintenance, transcription, cytoskeleton regulation, myogenesis and degradation of misfolded chaperone substrates (PubMed:12297501, PubMed:17696782, PubMed:23625928, PubMed:28445460, PubMed:16118278). Binds long polyubiquitin chains and trims them, while it has weak or no activity against chains of 4 or less ubiquitins (PubMed:17696782). 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 (By similarity). Interacts with key regulators of transcription and represses transcription: acts as a histone-binding protein that regulates transcription (PubMed:12297501). Regulates autophagy via the deubiquitination of 'Lys-402' of BECN1 leading to the stabilization of BECN1 (PubMed:28445460). {ECO:0000250|UniProtKB:Q9CVD2, ECO:0000269|PubMed:12297501, ECO:0000269|PubMed:16118278, ECO:0000269|PubMed:17696782, ECO:0000269|PubMed:23625928, ECO:0000269|PubMed:28445460}.
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GO - Biological processes (BP):
actin cytoskeleton organization [GO:0030036]; cellular response to heat [GO:0034605]; cellular response to misfolded protein [GO:0071218]; chemical synaptic transmission [GO:0007268]; intermediate filament cytoskeleton organization [GO:0045104]; microtubule cytoskeleton organization [GO:0000226]; mo...
actin cytoskeleton organization [GO:0030036]; cellular response to heat [GO:0034605]; cellular response to misfolded protein [GO:0071218]; chemical synaptic transmission [GO:0007268]; intermediate filament cytoskeleton organization [GO:0045104]; microtubule cytoskeleton organization [GO:0000226]; monoubiquitinated protein deubiquitination [GO:0035520]; nervous system development [GO:0007399]; nucleotide-excision repair [GO:0006289]; positive regulation of ERAD pathway [GO:1904294]; proteasome-mediated ubiquitin-dependent protein catabolic process [GO:0043161]; protein deubiquitination [GO:0016579]; protein K48-linked deubiquitination [GO:0071108]; protein K63-linked deubiquitination [GO:0070536]; protein localization to cytosolic proteasome complex involved in ERAD pathway [GO:1904379]; protein quality control for misfolded or incompletely synthesized proteins [GO:0006515]; regulation of cell-substrate adhesion [GO:0010810]; ubiquitin-dependent protein catabolic process [GO:0006511]
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GO - Molecular function (MF):
ATPase binding [GO:0051117]; identical protein binding [GO:0042802]; Lys48-specific deubiquitinase activity [GO:1990380]; Lys63-specific deubiquitinase activity [GO:0061578]; thiol-dependent deubiquitinase [GO:0004843]; ubiquitin protein ligase binding [GO:0031625]...
ATPase binding [GO:0051117]; identical protein binding [GO:0042802]; Lys48-specific deubiquitinase activity [GO:1990380]; Lys63-specific deubiquitinase activity [GO:0061578]; thiol-dependent deubiquitinase [GO:0004843]; ubiquitin protein ligase binding [GO:0031625]
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GO - Cellular component (CC):
cytoplasm [GO:0005737]; cytosol [GO:0005829]; mitochondrial matrix [GO:0005759]; mitochondrial membrane [GO:0031966]; nuclear inclusion body [GO:0042405]; nuclear matrix [GO:0016363]; nucleolus [GO:0005730]; nucleoplasm [GO:0005654]; nucleus [GO:0005634]; plasma membrane [GO:0005886]; synapse [GO:00...
cytoplasm [GO:0005737]; cytosol [GO:0005829]; mitochondrial matrix [GO:0005759]; mitochondrial membrane [GO:0031966]; nuclear inclusion body [GO:0042405]; nuclear matrix [GO:0016363]; nucleolus [GO:0005730]; nucleoplasm [GO:0005654]; nucleus [GO:0005634]; plasma membrane [GO:0005886]; synapse [GO:0045202]
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ATXN3
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Kaplan plot for ATXN3 in
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*NOTE: the Kaplan plots were collected from OncoLnc