Ataxin-3 (EC 3.4.19.12) (Machado-Joseph disease protein 1) (Spinocerebellar ataxia type 3 protein)
1_MESIF 6_ HEKQE 11_ GSLCA 16_ QHCLN 21_ NLLQG 26_ EYFSP 31_ VELSS 36_ IAHQL 41_ DEEER 46_ MRMAE 51_ GGVTS 56_ EDYRT 61_ FLQQP 66_ SGNMD 71_ DSGFF 76_ SIQVI 81_ SNALK 86_ VWGLE 91_ LILFN 96_ SPEYQ 101_ RLRID 106_ PINER 111_ SFICN 116_ YKEHW 121_ FTVRK 126_ LGKQW 131_ FNLNS 136_ LLTGP 141_ ELISD 146_ TYLAL 151_ FLAQL 156_ QQEGY 161_ SIFVV 166_ KGDLP 171_ DCEAD 176_ QLLQM 181_ IRVQQ 186_ MHRPK 191_ LIGEE 196_ LAQLK 201_ EQRVH 206_ KTDLE 211_ RVLEA 216_ NDGSG 221_ MLDED 226_ EEDLQ 231_ RALAL 236_ SRQEI 241_ DMEDE 246_ EADLR 251_ RAIQL 256_ SMQGS 261_ SRNIS 266_ QDMTQ 271_ TSGTN 276_ LTSEE 281_ LRKRR 286_ EAYFE 291_ KQQQK 296_ QQQQQ 301_ QQQQQ 306_ GDLSG 311_ QSSHP 316_ CERPA 321_ TSSGA 326_ LGSDL 331_ GDAMS 336_ EEDML 341_ QAAVT 346_ MSLET 351_ VRNDL 356_KTEGK
1: 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)