ATXN3 pAb

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. In response to misfolded substrate ubiquitination, mediates deubiquitination of monoubiquitinated STUB1/CHIP. Interacts with key regulators of transcription and represses transcription: acts as a histone-binding protein that regulates transcription.

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SPECIFICATIONS

Catalog Number

BS6285

Size

100ug/100ul

Applications

IHC, WB

Hosts

Rabbit

Reactivities

Hum, Mouse, Rat

Form

liquid

Purity

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).

Antigen

Recombinant full length Human ATXN3.

Species

ATXN3 pAb detects endogenous levels of ATXN3 protein.

Dilutions

WB: 1:500~1:2000\nIHC: 1:50~1:200

Swiss Prot

P54252

Storage Temp

Store at 4 degrees C short term. Aliquot and store at -20 degrees C long term. Avoid freeze-thaw cycles.

Concentration

1ug/ul

Storage Buffer

1 mg/ml in Phosphate buffered saline (PBS) with 0.05% sodium azide, approx. pH 7.2.

Additional Info

For research use only, not for use in diagnostic procedure.

Molecular Weight

~ 42 kDa

Alternative Names

AT3; Ataxin 3; Ataxin-3; ATX3; ATX3; ATXN3; EC 3.4.22.; JOS; Josephin; Machado Joseph disease (spinocerebellar ataxia 3; olivopontocerebellar ataxia 3; autosomal dominant; ataxin 3); Machado Joseph disease; Machado Joseph disease protein 1; Machado-Joseph disease protein 1; Machado-Joseph disease protein 1 homolog; MJD; MJD gene; MJD1; Olivopontocerebellar ataxia 3; OTTHUMP00000221583; OTTHUMP00000221585; OTTHUMP00000221586; OTTHUMP00000221587; OTTHUMP00000231995; OTTHUMP00000231997; Rsca3