Purified anti-Ataxin-3

Ataxin-3 is an ubiquitously expressed protein which contains a poly-glutamine tract. Genetically abnormal expansion of the poly-glutamine repeats results in accumulation of ubiquitinated ataxin-3 protein aggregates and leads to a neurodegenerative disorder, Machado–Joseph disease (MJD). The protein has ubiquitin interacting motifs and a N-terminal Cysteine residue (C14) responsible for its deubiquitinating activity. Ataxin-3 binds and hydrolyzes polyubiquitinated proteins, and interacts with key proteasome components, such as p97/VCP and p45, indicating that Ataxin-3 may play functional roles in ubiquitin-proteasome system. The protein also act as a transcriptional repressor when interacting with transcriptional activators and coactivators.

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SPECIFICATIONS

Catalog Number

650401

Size

25 microg

Applications

WB

Hosts

Mouse

Reactivities

Hum

Clone

1H9-2

Antigen

Partial Ataxin-3 recombinant protein

Gene Id

110616

Isotype

Mouse IgG1, kappa

Clonality

Monoclonal

Dilutions

Each lot of this antibody is quality control tested by Western blotting. Western blotting, suggested working dilution(s): Use 0.5 microg per ml antibody dilution buffer for each mini-gel. It is recommended that the reagent be titrated for optimal performance for each application.

Storage Temp

4 C

Concentration

0.5 mg/ml

Shipping Temp

Ambient RT

Storage Buffer

Upon receipt, store undiluted at 4C.

Alternative Names

ATX3, MJD, Machado-Joseph disease protein 1, SCA3, Spinocerebellar ataxia type 3 protein, JOS, josephin

Regulatory Status

RUO