ATXN3 Antibody

SKU: orb180467

Description

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.

Research Area

Neuroscience

Images & Validation

• Tested Applications: WB
• Dilution Range: WB:1:250-1:2,000
• Reactivity: Bovine, Canine, Donkey, Equine, Feline, Gallus, Goat, Guinea pig, Hamster, Human, Mouse, Other, Porcine, Primate, Rabbit, Rat, Sheep
• Application Notes:
  The antibody solution should be gently mixed before use.

Key Properties

• Host: Goat
• Clonality: Polyclonal
• Isotype: IgG
• Immunogen: Antigen: Purified recombinant peptide derived from within residues 120 aa to 250 aa of human ATXN3 produced in E. coli. Antigen Sequence: KEHWFTVRKLGKQWFNLNSLLTGPELISDTYLALFLAQLQQEGYSIFVVKGDLPDCEADQLLQMIRVQQMHRPKLIGEELAQLKEQRVHKTDLERVLEANDGSGMLDEDEEDLQRALALSRQEIDMEDEEADL
• Target: Ataxin 3
• Purification: Epitope affinity purified
• Conjugation: Unconjugated

Storage & Handling

• Storage: Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.
• Form/Appearance: Polyclonal antibody supplied as a 200 or 500 µl (3 mg/ml) aliquot in PBS, 20% glycerol and 0.05% sodium azide. This antibody is epitope-affinity purified from goat antiserum.
• Concentration: 1 mg/ml
• Expiration Date: 12 months from date of receipt.
• Disclaimer: For research use only

Alternative Names

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.

Quick Database Links

Gene Symbol

Ataxin 3