Anti-Ataxin 3/ATXN3 Antibody Picoband®
Primary Antibody supplied by Boster Bio Read more ›

Product Description
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.
Product information
| Supplier | Boster Bio |
|---|---|
| ABMIUM evidence status | ABMIUM Verified™ collaborator-tested data |
| Catalogue reference | PB9423 |
| Product category | Primary Antibodies |
| Reactive species | Human, Rat |
| Tested applications | ELISA, Flow Cytometry, IF/ICC, IHC, IHC-P, WB |
| Available conjugations | Unconjugated, Biotin, Cy3, Fluoro488, Fluoro550, Fluoro594, FITC, HRP, APC, PE, Fluoro647 |
| Gene name | ATXN3 |
| Protein name | Ataxin-3 |
| Specificity | No cross reactivity with other proteins. |
| 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. |
| Host | Rabbit |
| Clonality | Polyclonal |
| Isotype | Rabbit IgG |
| Purification | Immunogen affinity purified. |
| 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. |
| UniProt | P54252 |
| NCBI Gene ID | 4287 |
Available variants
| Conjugation | Size |
|---|---|
| Unconjugated | 100 μg/vial |
| Biotin | 100 μg/vial |
| Cy3 | 100 μg/vial |
| Fluoro488 | 100 μg/vial |
| Fluoro550 | 100 μg/vial |
| Fluoro594 | 100 μg/vial |
| FITC | 100 μg/vial |
| HRP | 100 μg/vial |
| APC | 100 μg/vial |
| PE | 100 μg/vial |
| Fluoro647 | 100 μg/vial |
Key Facts
| Target | Ataxin-3 |
| Also known as | AT3; ataxin 3; ATX3; ATX3; ATXN3; ATXN3; JOS; MJD; MJD1; SCA3 |
| Concentration | Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml. |
| Species reactivity | Human,Rat |
| Observed band | 42 kDa |
| Purity | No cross reactivity with other proteins. |
| Form / buffer | Lyophilized |
| Shelf life | 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. |
| Host species | Rabbit |
| Clonality | Polyclonal |
| Isotype | Rabbit IgG |
| Conjugation | Unconjugated|Biotin|Cy3|Fluoro488|Fluoro550|Fluoro594|FITC|HRP|APC|PE|Fluoro647 |
| Non-cross-reactive with | No cross-reactivity with other proteins |
| Tested applications |
ELISAFlow CytometryIF/ICCIHCIHC-PWB |
| Dilution range | 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 |
| 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. |
| Purification | Immunogen affinity purified. |
Reactivity & Application Validation
2 speciess| Species | WB | IHC | IHC-P | IHC-F | IF/ICC | FLOW CYTOMETRY | ELISA | IP |
|---|---|---|---|---|---|---|---|---|
| Human | ||||||||
| Rat |
Specifications
Immunohistochemistry (Paraffin-embedded Section), 0.5-1μg/ml, Human
Immunocytochemistry/Immunofluorescence, 5μg/ml, Human
Flow Cytometry (Fixed), 1-3μg/1x106 cells, Human
Storage & Stability
Compliance & Certifications
Not intended for diagnostic or therapeutic use.