Atxn3
entrez
60331
canonical
uniprot
O35815
canonical
ensembl
ENSRNOG00000005470
resolver
ensembl
ENSRNOG00055014517
resolver
ensembl
ENSRNOG00060004720
resolver
ensembl
ENSRNOG00065015145
resolver
ensp
ENSRNOP00000007505
resolver
ensp
ENSRNOP00000091691
resolver
ensp
ENSRNOP00000108385
resolver
ensp
ENSRNOP00055020411
resolver
ensp
ENSRNOP00060005977
resolver
ensp
ENSRNOP00065019676
resolver
enst
ENSRNOT00000007505
resolver
enst
ENSRNOT00000099444
resolver
enst
ENSRNOT00055024976
resolver
enst
ENSRNOT00060007983
resolver
enst
ENSRNOT00065025136
resolver
genesymbol
Atxn3
resolver
genesymbol_syn
Mjd
resolver
genesymbol_syn
Sca3
resolver
uniprot
A0A8I6GJ61
resolver
uniprot_entry
A0A8I6GJ61_RAT
resolver
uniprot_entry
ATX3_RAT
resolver
amino_acid_sequence
MESIFHEKQEGSLCAQHCLNNLLQGEYFSPVELSSIAHQLDEEERLRMAEGGVTSEDYRTFLQQPSGNMDDSGFFSIQVISNALKVWGLELILFNSPEYQRLRIDPINERSFICNYKEHWFTVRKLGKQWFNLNSLLTGPELISDTYLALFLAQLQQEGYSIFVVKGDLPDCEADQLLQMIKVQQMHRPKLIGEELAHLKEQSALKADLERVLEAADGPGMFDDDEDDLQRALAMSRQEIDMEDEEADLRRAIQLSMQGSSRGMCEDSPQTSSTDLSSEELRKRREAYFEKQQHQQQEADRPGYLSYPCERPTTSSGGLRSNQAGNAMSEEDVLRATVTVSLETAKDSLKAERKK
uniprot
proteins
ec_number
3.4.19.12
uniprot
proteins
function
FUNCTION: Deubiquitinating enzyme involved in protein homeostasis maintenance, transcription, cytoskeleton regulation, myogenesis and degradation of misfolded chaperone substrates (PubMed:17696782). Binds long polyubiquitin chains and trims them, while it has weak or no activity against chains of 4 or less ubiquitins (By similarity). 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 (By similarity). 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 (By similarity). Regulates autophagy via the deubiquitination of 'Lys-402' of BECN1 leading to the stabilization of BECN1 (By similarity). {ECO:0000250|UniProtKB:P54252, ECO:0000250|UniProtKB:Q9CVD2, ECO:0000269|PubMed:17696782}.
uniprot
proteins
mass_dalton
40446
uniprot
proteins
mutagenesis
MUTAGEN 14; /note="C->A,S: Loss of enzyme activity."; /evidence="ECO:0000269|PubMed:17696782"; MUTAGEN 119; /note="H->A: Loss of enzyme activity."; /evidence="ECO:0000269|PubMed:17696782"; MUTAGEN 134; /note="N->A: Loss of enzyme activity."; /evidence="ECO:0000269|PubMed:17696782
uniprot
proteins
post_translational_modification
PTM: Monoubiquitinated by UBE2W, possibly leading to activate the deubiquitinating enzyme activity (By similarity). {ECO:0000250|UniProtKB:P54252}.
uniprot
proteins
pubmed
10732811
uniprot
proteins
pubmed
12486728
uniprot
proteins
pubmed
17696782
uniprot
proteins
pubmed
22673903
uniprot
proteins
sequence_length
355
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Nucleus matrix {ECO:0000250|UniProtKB:P54252}. Nucleus {ECO:0000250|UniProtKB:P54252}. Lysosome membrane {ECO:0000250|UniProtKB:P54252}; Peripheral membrane protein {ECO:0000250|UniProtKB:P54252}. Note=Predominantly nuclear, but not exclusively, inner nuclear matrix. Recruited to lysosomal membrane in response to amino acid deprivation by the RagA/RRAGA-RagB/RRAGB complex. {ECO:0000250|UniProtKB:P54252}.
uniprot
proteins