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Conditions of optimal ATXN3 Antibody performance should be determined experimentally by the investigator.
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Fax: 1-617-606-5019
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Or leave a message with a formal purchase
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Introduction
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.
PA006689-PA1846: ATXN3 Polyclonal Antibody
Rabbit Polyclonal Antibody.
Applications: WB 0.1-0.5 ug/ml; IHC-P 0.5-1 ug/ml.
Reactivity: Human, Rat, Mouse.
Immunogen: A synthetic peptide corresponding to a sequence at the N-terminal of human ATXN3, identical to the related rat and mouse sequences.
Purification: Immunogen affinity purified.
Form of Antibody: Lyophilized.
Isotype: Rabbit IgG.
Storage/Stability: Stable for 1 year at -20°C and 3 months at 4°C. For maximum recovery of the product, centrifuge the original vial after thawing and before removing the cap. Aliquot to avoid repeated freezing and thawing.
ATXN3 Protein:
Recombinant Human ATXN3 Protein
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