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| Reactivity: | Human, Mouse, Rat |
| Applications: | WB, IHC, 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 1:500-1:1000; IHC 1:50-1:200; IF/IC 1:50-1:200 |
| Purification Method: | Affinity purification |
| Concentration: | 1.61 mg/ml |
| Buffer: | PBS with 0.05% proclin300, 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 (A1243) 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. Detection: ECL Basic Kit (RM00020). Exposure time: 10s. |
 | Immunohistochemistry analysis of paraffin-embedded Human esophageal cancer using Ataxin-3 (ATXN3) Rabbit pAb (A1243) at dilution of 1:100 (40x lens). High pressure antigen retrieval performed with 0.01M Citrate Bufferr (pH 6.0) prior to IHC staining. |
 | Immunohistochemistry analysis of paraffin-embedded Mouse lung using Ataxin-3 (ATXN3) Rabbit pAb (A1243) at dilution of 1:100 (40x lens). High pressure antigen retrieval performed with 0.01M Citrate Bufferr (pH 6.0) prior to IHC staining. |
 | Immunohistochemistry analysis of paraffin-embedded Rat kidney using Ataxin-3 (ATXN3) Rabbit pAb (A1243) at dilution of 1:100 (40x lens). High pressure antigen retrieval performed with 0.01M Citrate Bufferr (pH 6.0) prior to IHC staining. |
 | Confocal immunofluorescence analysis of U-2 OS cells using Ataxin-3 (ATXN3) Rabbit pAb (A1243) at dilution of 1:100. Blue: DAPI for nuclear staining. |
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