Anti-Ataxin 3/ATXN3 Antibody Picoband®

SKU: PB9423

Size: 100 μg

Storage: Store at -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freeze-thaw cycles.

Form: Lyophilized

Reactivity: Human,Rat

Applications: Flow Cytometry,IF,IHC,ICC,WB

Application Details: Western blot, 0.1-0.5μg/ml, Human, Rat
Immunohistochemistry (Paraffin-embedded Section), 0.5-1μg/ml, Human
Immunocytochemistry/Immunofluorescence, 5μg/ml, Human
Flow Cytometry (Fixed), 1-3μg/1x10⁶ cells, Human

Gene Name: ATXN3

Specificity: No cross reactivity with other proteins.

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 ranges 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.

Immunogen: A synthetic peptide corresponding to a sequence at the C-terminus of human Ataxin 3, different from the related mouse and rat sequences by two amino acids.

Clonality: Polyclonal

Contents: Each vial contains 4 mg Trehalose, 0.9 mg NaCl and 0.2 mg Na2HPO4.

Purification: Immunogen affinity purified.

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

Reference: 1. Burnett, B., Li, F., Pittman, R. N. The polyglutamine neurodegenerative protein ataxin-3 binds polyubiquitylated proteins and has ubiquitin protease activity. Hum. Molec. Genet. 12: 3195-3205, 2003. 2. Paulson, H. L., Das, S. S., Crino, P. B., Perez, M. K., Patel, S. C., Gotsdiner, D., Fischbeck, K. H., Pittman, R. N.Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain.Ann. Neurol. 41: 453-462, 1997.

Uniprot ID: P54252

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate: This is the unconjugated antibody. It is also available with 10 conjugation options, including Biotin, Cy3, Fluoro488, Fluoro550, Fluoro594, FITC, HRP, APC, PE, and Fluoro647. A carrier-free (BSA-free) version is also available. Additional charges may apply for some options. Please contact us for a quote

Cross Reactivity: No cross-reactivity with other proteins

Isotype: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 42 kDa

Calculated Molecular Weight: 41781 MW

Gene ID: 4287

Protein Name: Ataxin-3

Gene Full Name: ataxin 3

Synonyms: AT3; ataxin 3; ATX3; ATX3; ATXN3; ATXN3; JOS; MJD; MJD1; SCA3