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Catalog #
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AB10089
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Category
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Antibodies
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Family
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Neurobiology
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Source
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Goat
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Application
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WB
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Reactivity
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Human, Rat, Mouse, Monkey, Canine
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Description
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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.
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Alternative names
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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.
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Gene Identifier/Accession#
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Gene ID: 4287, ENSG00000066427
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Form
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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.
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Concentration
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3.00 mg/ml
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Isotype
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IgG
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Clonality
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Polyclonal
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Conjugation
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Unconjugated
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Immunogen
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Purified recombinant peptide derived from within residues 120 aa to 250 aa of human ATXN3 produced in E. coli.
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Antigen Sequence
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KEHWFTVRKLGKQWFNLNSLLTGPELISDTYLALFLAQLQQEGYSIFVVKGDLPDCEADQLLQMIRVQQMHRPKLIGEELAQLKEQRVHKTDLERVLEANDGSGMLDEDEEDLQRALALSRQEIDMEDEEADL
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Specificity
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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.
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Storage
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For extended storage, store in -20 C freezer.
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Special instructions
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The antibody solution should be gently mixed before use.
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Application
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Values
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WB
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1:250-1:2,000
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Sample
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WB
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IHC (F)
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IHC (P)
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IF
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ELISA
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Human
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+++
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ND
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ND
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ND
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ND
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Rat
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+++
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ND
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ND
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ND
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ND
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Mouse
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+++
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ND
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ND
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ND
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ND
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Canine
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+++
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ND
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ND
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ND
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ND
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Monkey
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+++
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ND
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ND
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ND
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ND
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Anti-ATXN3 Ab at 1/500 dilution; lysates at 100 µg per lane; rabbit polyclonal to goat IgG (HRP) at 1/10,000 dilution;
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