Atxn3
entrez
110616
canonical
uniprot
Q9CVD2
canonical
ensembl
ENSMUSG00000021189
resolver
ensp
ENSMUSP00000021606
resolver
ensp
ENSMUSP00000125082
resolver
ensp
ENSMUSP00000125378
resolver
ensp
ENSMUSP00000200385
resolver
ensp
ENSMUSP00000200392
resolver
enst
ENSMUST00000021606
resolver
enst
ENSMUST00000160251
resolver
enst
ENSMUST00000161011
resolver
genesymbol
Atxn3
resolver
genesymbol_syn
Mjd
resolver
uniprot
E9Q717
resolver
uniprot
F7DEL3
resolver
uniprot
Q546X9
resolver
uniprot
Q5M8S1
resolver
uniprot
Q810M8
resolver
uniprot_entry
ATX3_MOUSE
resolver
uniprot_entry
E9Q717_MOUSE
resolver
uniprot_entry
F7DEL3_MOUSE
resolver
uniprot_entry
Q546X9_MOUSE
resolver
uniprot_entry
Q5M8S1_MOUSE
resolver
uniprot_entry
Q810M8_MOUSE
resolver
amino_acid_sequence
MESIFHEKQEGSLCAQHCLNNLLQGEYFSPVELSSIAHQLDEEERLRMAEGGVTSEDYRTFLQQPSGNMDDSGFFSIQVISNALKVWGLELILFNSPEYQRLRIDPINERSFICNYKEHWFTVRKLGKQWFNLNSLLTGPELISDTYLALFLAQLQQEGYSIFVVKGDLPDCEADQLLQMIKVQQMHRPKLIGEELAHLKEQSALKADLERVLEAADGSGIFDEDEDDLQRALAISRQEIDMEDEEADLRRAIQLSMQGSSRSMCENSPQTSSPDLSSEELRRRREAYFEKQQQQQQEVDRPGPLSYPRERPTTSSGGRRSDQGGDAVSEEDMLRAAVTMSLETAKDNLKAERKK
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 (By similarity). 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 (PubMed:21855799). 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 (PubMed:28445460). {ECO:0000250|UniProtKB:P54252, ECO:0000269|PubMed:21855799, ECO:0000269|PubMed:28445460}.
uniprot
proteins
mass_dalton
40533
uniprot
proteins
mutagenesis
MUTAGEN 14; /note="C->A: Abolishes deubiquitinating activity."; /evidence="ECO:0000269|PubMed:21855799
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
12486728
uniprot
proteins
pubmed
15345747
uniprot
proteins
pubmed
16141072
uniprot
proteins
pubmed
17242355
uniprot
proteins
pubmed
21183079
uniprot
proteins
pubmed
21855799
uniprot
proteins
pubmed
28445460
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