Ataxin 3 polyclonal, anti-human, mouse, rat

Quantity: 100 µg

Background:
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.

Description:
Rabbit IgG polyclonal antibody for Ataxin-3 (ATXN3) detection. Tested with WB, IHC-P in Human, Mouse, Rat.

Synonyms:
AT3 antibody, Ataxin 3 antibody, Ataxin-3 antibody, ATX3 antibody, ATX3_HUMAN antibody, ATXN3 antibody, EC 3.4.22. antibody, JOS antibody, Josephin antibody, Machado Joseph disease(spinocerebellar ataxia 3, olivopontocerebellar ataxia 3, autosomal dominant, ataxin 3) antibody, Machado Joseph disease antibody, Machado Joseph disease protein 1 antibody, Machado-Joseph disease protein 1 antibody, Machado-Joseph disease protein 1 homolog antibody, MJD antibody, MJD gene antibody, MJD1 antibody, Olivopontocerebellar ataxia 3 antibody, OTTHUMP00000221583 antibody, OTTHUMP00000221585 antibody, OTTHUMP00000221586 antibody, OTTHUMP00000221587 antibody, OTTHUMP00000231995 antibody, OTTHUMP00000231997 antibody, Rsca3 antibody, SCA3 antibody, SCA3 gene antibody, Spinocerebellar ataxia type 3 protein antibody

Isotype: Rabbit polyclonal IgG

Immunogen:
A synthetic peptide corresponding to a sequence at the N-terminus of human Ataxin 3(7-21aa EKQEGSLCAQHCLNN), identical to the related rat and mouse sequences.A synthetic peptide corresponding to a sequence at the N-terminus of human Ataxin 3(7-21aa EKQEGSLCAQHCLNN), identical to the related rat and mouse sequences.

Form: Lyophilized; Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg Thimerosal, 0.05mg NaN3.

Specificity:
No cross reactivity with other proteins.

Reactivity: Reacts with: human, rat .
Predicted to work with: mouse.

Applications: WB, IHC-P

Reconstitution:
Add 0.2ml of distilled water will yield a concentration of 500ug/ml.

Storage: At -20˚C for one year. After reconstitution, at 4˚C for one month. It can also be aliquotted and stored frozen at -20˚C for a longer time. Avoid repeated freezing and thawing.