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You are here:Home » Antibodies » Abs to Ataxin » Anti -Ataxin 3 (Ataxin-3, Machado-Joseph Disease Protein 1, Spinocerebellar Ataxia Type 3 Protein, ATXN3, ATX3, MJD, MJD1, SCA3)

Anti -Ataxin 3 (Ataxin-3, Machado-Joseph Disease Protein 1, Spinocerebellar Ataxia Type 3
Protein, ATXN3, ATX3, MJD, MJD1, SCA3)

Pricing

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Specifications

Clone Host Grade Applications
Polyclonal Rabbit Affinity Purified B IH
ATXN3(ataxin 3), also known as AT3, MJD GENE, MJD1, SCA3 GENE, ATX3, JOS, Spinocerebellar ataxia-3, Machado-Joseph disease protein 1, is a protein that in humans is encoded by the ATXN3 gene. ATXN3 range in size from 360 to 374 amino acids. Using Northern blot analysis showed that ATXN3 mRNA was ubiquitously expressed in human tissues. They detected at least 4 ATXN3 transcripts of 1.4, 1.8, 4.5, and 7.5 kb and suggested that the different mRNA species probably result from differential splicing and polyadenylation.Machado-Joseph disease, also known as spinocerebellar ataxia-3, is an autosomal dominant neurologic disorder. The protein encoded by the ATXN3 gene contains (CAG)n repeats in the coding region, and the expansion of these repeats from the normal 13-36 to 68-79 is the cause of Machado-Joseph disease. There is an inverse correlation between the age of onset and CAG repeat numbers. Alternatively spliced transcript variants encoding different isoforms have been described for this gene. Ataxin-3 interacted with 2 human homologs of the yeast DNA repair protein RAD23, HHR23A (RAD23A) and HHR23B (RAD23B). Both normal and mutant ataxin-3 proteins interacted with the ubiquitin-like domain at the N terminus of the HHR23 proteins, which is a motif important for nucleotide excision repair. However, in HEK 293 cells, HHR23A was recruited to intranuclear inclusions formed by the mutant ataxin-3 through its interaction with ataxin-3.
Catalog #144213
ApplicationsSuitable for use in Western Blot and Immunohistochemistry ([araffin). Other applications not tested.
Recommended DilutionImmunohistochemistry (paraffin sections): 0.5 - 1ug/ml. Perform HIER by boiling paraffin sections in 10mM citrate buffer, pH 6.0, for 20 mins.
Western Blot: 0.1 - 0.5ug/ml
Optimal dilutions to be determined by the researcher.
Storage and StabilityLyophilized powder may be stored at -20°C. Stable for 12 months after receipt at -20°C. Reconstitute with sterile ddH2O. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Reconstituted product is stable for 12 months at -20°C. For maximum recovery of product, centrifuge the original vial after thawing and prior to rem
Clone TypePolyclonal
IsotypeIgG
HostRabbit
SourceHuman
Concentration~0.5mg/ml (after reconstitution)
FormSupplied as a lyophilized powder. Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg Thimerosal, 0.05mg NaN3. Reconstitute to 0.5mg/ml with 200ul distilled water.
PurityPurified by immunoaffinity chromatography.
ImmunogenA synthetic peptide corresponding to a sequence at the N-terminal of human ATXN3, identical to the related rat and mouse sequences.
SpecificityRecognizes human and rat ATXN3. Predicted to cross-react with mouse. No crossreactivity with other proteins.
Important NoteThis product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications without the expressed written authorization of United States Biological.


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