| Post Translational Modifications | Monoubiquitinated N-terminally by UBE2W, possibly leading to activate the deubiquitinating enzyme activity. |
| Function | Deubiquitinating enzyme involved in protein homeostasis maintenance, transcription, cytoskeleton regulation, myogenesis and degradation of misfolded chaperone substrates. Binds long polyubiquitin chains and trims them, while it has weak or no activity against chains of 4 or less ubiquitins. 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. Interacts with key regulators of transcription and represses transcription: acts as a histone-binding protein that regulates transcription. 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. Regulates autophagy via the deubiquitination of 'Lys-402' of BECN1 leading to the stabilization of BECN1. |
| Protein Name | Ataxin-3Machado-Joseph Disease Protein 1Spinocerebellar Ataxia Type 3 Protein |
| Database Links | Reactome: R-HSA-5689877Reactome: R-HSA-9615017 |
| Cellular Localisation | Nucleus MatrixNucleusLysosome MembranePeripheral Membrane ProteinPredominantly NuclearBut Not ExclusivelyInner Nuclear MatrixRecruited To Lysosomal Membrane In Response To Amino Acid Deprivation By The Raga/Rraga-Ragb/Rragb Complex |
| Alternative Protein Names | Ataxin-3 proteinMachado-Joseph Disease Protein 1 proteinSpinocerebellar Ataxia Type 3 Protein proteinATXN3 proteinATX3 proteinMJD proteinMJD1 proteinSCA3 protein |
Information sourced from Uniprot.org
