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 (By similarity). 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 .

P54252

4287

Ataxin-3, Machado-Joseph disease protein 1, Spinocerebellar ataxia type 3 protein, ATXN3, ATX3, MJD, MJD1, SCA3

Human

WB,IHC,FC

WB 1:1000
IHC 1:100-500
FC 1:10-50

42 kD

Rabbit

Polyclonal

Rabbit IgG

This antibody is purified by peptide affinity purification.

PBS (pH7.4) with 0.09% (W/V) sodium azide.

Store at -20°C. Stable for 12 months from date of receipt.Do not aliquot the antibody.Avoid freeze / thaw cycle.