Anti-ATXN3
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Catalog #
AB0089
Category
Antibodies
Family
Neurobiology
Source
Goat
Application
WB
Reactivity
Human, Rat, Mouse, Monkey, Canine
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.
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.
Gene Identifier/Accession#
Gene ID: 4287, ENSG00000066427
Form
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
3.00 mg/ml
Isotype
IgG
Clonality
Polyclonal
Conjugation
Unconjugated
Immunogen
Purified recombinant peptide derived from within residues 120 aa to 250 aa of human ATXN3 produced in E. coli.
Antigen Sequence
KEHWFTVRKLGKQWFNLNSLLTGPELISDTYLALFLAQLQQEGYSIFVVKGDLPDCEADQLLQMIRVQQMHRPKLIGEELAQLKEQRVHKTDLERVLEANDGSGMLDEDEEDLQRALALSRQEIDMEDEEADL
Specificity
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 AB10089.
Storage
For continuous use, store at 2-8 C for one-two days. For extended storage, store in -20 C freezer. Working dilution samples should be discarded if not used within 12 hours.
Special instructions
The antibody solution should be gently mixed before use.

Usage

Application Values
WB 1:250-1:2,000

Reactivity Chart

Sample WB IHC (F) IHC (P) IF ELISA
Human +++ ND ND ND ND
Rat +++ ND ND ND ND
Mouse +++ ND ND ND ND
Canine +++ ND ND ND ND
Monkey +++ ND ND ND ND

Images

Anti-ATXN3 Ab at 1/1,000 dilution; lysates at 50 µg per lane; rabbit polyclonal to goat IgG (HRP) at 1/10,000 dilution;

375 €

Quantity / Unit Size

600 µg
1.5 mg
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