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| Reactivity: | Human, Mouse |
| Applications: | WB, IF/IC, ELISA |
| Host Species: | Rabbit |
| Isotype: | IgG |
| Clonality: | Polyclonal antibody |
| Gene Name: | ataxin 3 |
| Gene Symbol: | ATXN3 |
| Synonyms: | AT3; JOS; MJD; ATX3; MJD1; SCA3; Ataxin-3 (ATXN3) |
| Gene ID: | 4287 |
| UniProt ID: | P54252 |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-361 of human Ataxin-3 (ATXN3) (NP_004984.2). |
| Dilution: | WB 1:500-1:2000; IF/IC 1:50-1:200 |
| Purification Method: | Affinity purification |
| Concentration: | 0.15 mg/ml |
| Buffer: | PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| Storage: | Store at -20°C. Avoid freeze/thaw cycles. |
| Documents: | Manual-ATXN3 antibody |
Background
Machado-Joseph disease, also known as spinocerebellar ataxia-3, is an autosomal dominant neurologic disorder. The protein encoded by this gene contains (CAG)n repeats in the coding region, and the expansion of these repeats from the normal 12-44 to 52-86 is one cause of Machado-Joseph disease. There is a negative correlation between the age of onset and CAG repeat numbers. Alternatively spliced transcript variants encoding different isoforms have been described for this gene.
Images
 | Western blot analysis of various lysates using Ataxin-3 (ATXN3) Rabbit pAb (A12992) at 1:1000 dilution. Secondary antibody: HRP-conjugated Goat anti-Rabbit IgG (H+L) (AS014) at 1:10000 dilution. Lysates/proteins: 25μg per lane. Blocking buffer: 3% nonfat dry milk in TBST. |
 | Immunofluorescence analysis of HeLa cells using Ataxin-3 (ATXN3) Rabbit pAb (A12992). Secondary antibody: Cy3-conjugated Goat anti-Rabbit IgG (H+L) (AS007) at 1:500 dilution. Blue: DAPI for nuclear staining. |
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