| 克隆类型 ： |
Rabbit Polyclonal IgG |
|
免 疫 原 ： |
peptide corresponding to a sequence at the N-terminus of human Ataxin 3. |
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纯化方式 ： |
Immunogen affinity purified |
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缓 冲 液 ： |
PBS, pH 7.4(1%BSA and 0.1% Sodium azide) |
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产品描述 ： |
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 range 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. |
| 适用物种 ： |
Hu, Mo, Rat |
| 应用说明 ： |
WB: 1:200-1:1000 IHC: 1:500-1:1000 |
| 别 名 ： |
Ataxin-3;3.4.19.12;Machado-Joseph disease protein 1;Spinocerebellar ataxia type 3 protein;ATXN3;ATX3, MJD, MJD1, SCA3. |
| 保存条件 ： |
Store at -20°C for one year. Avoid repeated freeze-thaw cycles. At 4°C for 2-3 weeks after reconstituted. |
| 标 记 物 ： |
PLLABS公司可供应生物素、辣根过氧化物酶、荧光素标记抗体 |
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产品说明 ： |
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