Goat anti-ATXN3 Polyclonal antibody

Goat polyclonal antibody to ATXN3. This protein appears to be a component of the ubiquitin proteasome system and has deubiquitinase activity. It may also have roles in neuroprotection, protein homeostasis maintenance, transcriptional and cytoskeleton regulation, myogenesis and degradation of misfolded chaperone substrates. The protein contains Qn repeats in the coding region, and the expansion of these repeats from the normal 13-36 to 68-79 is one cause of Machado-Joseph disease also known as spinocerebellar ataxia-3, an autosomal dominant neurologic disorder.

Product Specifications

CAS Number

9007-83-4

Specifications

Detects levels of ATXN3 protein in transfected cells with GFP-ATXN3 and endogenous levels in U118, SH-SY5Y, At-T20 and hTERT cell lysates by Western blot. This Ab is similar to AB0089.

Product Name Alternative

AT3, ATX3, ataxin-3, ataxin 3 variant h, autosomal dominant ataxin 3, ataxin 3 variant m, ataxin 3 variant ref, JOS, josephin, Machado-Joseph, Machado-Joseph disease protein 1, MJD, MJD1, SCA3, spinocerebellar ataxia 3, olivopontocerebellar ataxia 3, spinocerebellar ataxia type 3 protein antibody.

UNSPSC Description

Ataxin 3

Volume

200 µL

Gene ID

ENSG00000066427

Accession Number

ENSG00000066427

Host

Goat

Antigen Species

Human

Reactivity

Human, mouse, rat, bovine, canine, chicken/avian, donkey, feline, goat, guinea pig, hamster, horse, porcine, rabbit, sheep, simian, other

Immunogen

Purified recombinant peptide derived from within residues 120 aa to 250 aa of human ATXN3 produced in E. coli.

Target Antigen

Purified recombinant peptide derived from within residues 120 aa to 250 aa of human ATXN3 produced in E. coli.

Immunogen Type

Recombinant protein

Target

Anti-ATXN3

Clonality

Polyclonal

Isotype

IgG

Conjugation

Unconjugated

Type

Primary

Sequence

KEHWFTVRKLGKQWFNLNSLLTGPELISDTYLALFLAQLQQEGYSIFVVKGDLPDCEADQLLQMIRVQQMHRPKLIGEELAQLKEQRVHKTDLERVLEANDGSGmLDEDEEDLQRALALSRQEIDMEDEEADL

Applications

Purification

Epitope affinity purified

Concentration

3 mg/mL

Dilution

WB:1:250-1:2,000

Format

Preservatives-Free

Form

Polyclonal antibody supplied as a 200 or 500 µl (3 mg/mL) aliquot in PBS. It does not contain preservatives. Glycerol and azide FREE. This antibody is epitope-affinity purified from goat antiserum.

Buffer

PBS; no preservatives; glycerol and azide FREE

Storage Conditions

For continuous use, store at 2-8 deg;C for one-two days. For extended storage, store in -20 deg;C freezer. Working dilution samples should be discarded if not used within 12 hours.