Anti-Ataxin 3 Antibody | Boster Immunoleader PB9423 product information

product summary

request information :

company name :

Boster Immunoleader

product type :

antibody

product name :

Anti-Ataxin 3 Antibody

catalog :

PB9423

quantity :

100 ug/vial

price :

240 USD

clonality :

polyclonal

host :

rabbit

reactivity :

human, rat

application :

western blot, immunohistochemistry - paraffin section

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product information

Product Name :

Anti-Ataxin 3 Antibody

Catalog Number :

PB9423

Clonality :

Polyclonal

Description short :

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

Price :

240 USD

Ig type :

Rabbit IgG

Size :

100 ug/vial

Form :

Lyophilized

Specificity :

No cross reactivity with other proteins.

Reactivity :

Reacts with: human, rat

Applications :

WB,IHC-P

Western blot :

Concentration: 0.1-0.5μg/ml; Tested Species: Human, Rat

Immunohistochemistry(Paraffin-embedded S ... :

Concentration: 0.5-1μg/ml; Tested Species: Human

Notes :

Tested Species: In-house tested species with positive results. By Heat: Boiling the paraffin sections in 10mM citrate buffer, pH6.0, for 20mins is required for the staining of formalin/paraffin sections. Other applications have not been tested. Optimal dilutions should be determined by end users.

Immunogen :

A synthetic peptide corresponding to a sequence at the C-terminus of human Ataxin 3 (226-254aa EEDLQRALALSRQEIDMEDEEADLRRAIQ), different from the related mouse and rat sequences by two amino acids.

Contents :

Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg NaN3.

Purification :

Immunogen affinity purified.

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.

Relevant detection systems :

Boster provides a series of assays reacted with primary antibodies. Antibody can be supported by chemiluminescence kit EK1002 in WB, supported by SA1022 in IHC(P).

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.

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.

Gene Name :

ATXN3

Protein Name :

Ataxin-3

Gene Full Name :

ataxin 3

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

Uniprot ID :

P54252

more info or order :

Boster Immunoleader product webpage