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.

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

• Antibody Type: Primary Antibody
• 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

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.
• Buffer/Preservatives: PBS, 20% glycerol and 0.05% sodium azide
• Concentration: 1 mg/ml
• 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.

Western blot analysis of SH-SY5Y cell line lysate using ATXN3 antibody.

Quick Database Links

Gene Symbol

Ataxin 3