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Overview

Uniprot IDP54252
Protein NameAtaxin-3
Gene NameATXN3
OrganismHomo sapiens

Kla Sites from experimental identification

Position Flanking peptide
206 LKEQRVHKTDLERVL

Function

Deubiquitinating enzyme involved in protein homeostasis maintenance, transcription, cytoskeleton regulation, myogenesis and degradation of misfolded chaperone substrates (PubMed:12297501, PubMed:16118278, PubMed:17696782, PubMed:23625928, PubMed:28445460, PubMed:33157014). 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). Acts as a negative regulator of mTORC1 signaling in response to amino acid deprivation by mediating deubiquitination of RHEB, thereby promoting RHEB inactivation by the TSC-TBC complex (PubMed:33157014). Regulates autophagy via the deubiquitination of 'Lys-402' of BECN1 leading to the stabilization of BECN1 (PubMed:28445460)

Protein Sequence

10 MESIFHEKQE 20 GSLCAQHCLN 30 NLLQGEYFSP 40 VELSSIAHQL 50 DEEERMRMAE 60 GGVTSEDYRT 70 FLQQPSGNMD 80 DSGFFSIQVI 90 SNALKVWGLE 100 LILFNSPEYQ 110 RLRIDPINER 120 SFICNYKEHW 130 FTVRKLGKQW 140 FNLNSLLTGP 150 ELISDTYLAL 160 FLAQLQQEGY 170 SIFVVKGDLP 180 DCEADQLLQM 190 IRVQQMHRPK 200 LIGEELAQLK 210 EQRVHKTDLE 220 RVLEANDGSG 230 MLDEDEEDLQ 240 RALALSRQEI 250 DMEDEEADLR 260 RAIQLSMQGS 270 SRNISQDMTQ 280 TSGTNLTSEE 290 LRKRREAYFE 300 KQQQKQQQQQ 310 QQQQQGDLSG 320 QSSHPCERPA 330 TSSGALGSDL 340 GDAMSEEDML 350 QAAVTMSLET 360 VRNDLKTEGK K

Gene Ontology

Classification GO ID Description
Cellular Component GO:0005737 cytoplasm
Molecular Function GO:1990380 K48-linked deubiquitinase activity
Molecular Function GO:0061578 K63-linked deubiquitinase activity
Molecular Function GO:0031625 ubiquitin protein ligase binding
Biological Process GO:0030036 actin cytoskeleton organization
Biological Process GO:0034198 cellular response to amino acid starvation
Biological Process GO:0034605 cellular response to heat
Biological Process GO:0071218 cellular response to misfolded protein
Biological Process GO:0007268 chemical synaptic transmission
Biological Process GO:0006351 DNA-templated transcription
Biological Process GO:0045104 intermediate filament cytoskeleton organization
Biological Process GO:0000226 microtubule cytoskeleton organization
Biological Process GO:0035520 monoubiquitinated protein deubiquitination
Biological Process GO:1904262 negative regulation of TORC1 signaling
Biological Process GO:0007399 nervous system development
Biological Process GO:0006289 nucleotide-excision repair
Biological Process GO:1904294 positive regulation of ERAD pathway
Biological Process GO:2000060 positive regulation of ubiquitin-dependent protein catabolic process
Biological Process GO:0043161 proteasome-mediated ubiquitin-dependent protein catabolic process
Biological Process GO:0016579 protein deubiquitination
Biological Process GO:0071108 protein K48-linked deubiquitination
Biological Process GO:0070536 protein K63-linked deubiquitination
Biological Process GO:1904327 protein localization to cytosolic proteasome complex
Biological Process GO:0006515 protein quality control for misfolded or incompletely synthesized proteins
Biological Process GO:0010810 regulation of cell-substrate adhesion
Biological Process GO:0006511 ubiquitin-dependent protein catabolic process
Cellular Component GO:0005829 cytosol
Cellular Component GO:0005765 lysosomal membrane
Cellular Component GO:0005759 mitochondrial matrix
Cellular Component GO:0031966 mitochondrial membrane
Cellular Component GO:0042405 nuclear inclusion body
Cellular Component GO:0016363 nuclear matrix
Cellular Component GO:0005654 nucleoplasm
Cellular Component GO:0005634 nucleus
Cellular Component GO:0045202 synapse
Molecular Function GO:0051117 ATPase binding
Molecular Function GO:0004843 cysteine-type deubiquitinase activity
Molecular Function GO:0042802 identical protein binding

Reference

PMID: N/A