Specifications
- Antibody type:Primary
- Antigen name:ataxin 3
- Antigen symbol:ATXN3
- Clonality:Polyclonal
- Host:Rabbit
- ImmunoChemistry:Yes
- Isotype:IgG
- Reactivity:Human,Rat
- Western blot:Yes
- Form:Lyophilized
- Gene ID:4287
- Antigen synonyms:MJD1|AT3|MJD|JOS|SCA3|ATX3
- UniProtKB:P54252
- Storage temperature: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.
- 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.
- Purification:Immunogen affinity purified.
- Size:100 µg/vial
- Pk:100 µG
Specifications
About this item
Rabbit IgG polyclonal antibody for Ataxin-3(ATXN3) detection. Tested with WB, IHC-P in Human;Rat.
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.
Add 0.2ml of distilled water will yield a concentration of 500ug/ml.
Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg NaN3.
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.
Type: Primary
Antigen: CCNA2
Clonality: Polyclonal
Clone:
Conjugation:
Epitope:
Host: Rabbit
Isotype: IgG
Reactivity: Human, Rat