产品货号：
Z12021
中文名称：
ATX3/ATXN3抗体
英文名称：
Anti-ATX3/ATXN3 Antibody
产品规格：
50μl|100μl|150μl
发货周期：
1～3天
产品价格：
询价
| 抗体名称 | Anti-ATX3/ATXN3 Antibody |
| 指标别名 | AT3;ataxin 3;ATX3;ATX3;ATXN3;ATXN3;JOS;MJD;MJD1;SCA3 |
| 克隆性 | Polyclonal |
| 检验物种 | human,rat |
| 应用范围 | WB,IHC,ICC/IF,FCM |
| 基因名称 | ATXN3 |
| 抗体来源 | Rabbit |
| 抗体类型 | IgG |
| 免疫原 | 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. |
| 实际分子量 | 42KD |
| 成分 | 500μg/mL antibody with PBS，0.02% NaN3,1mg BSA and 50% glycerol. |
| 纯化方式 | Immunogen affinity purified. |
| 浓度 | 500μg/mL |
| 产品形态 | 溶液 |
| 保存条件 | 12 months from date of receipt，-20℃ as supplied.6 months 2 to 8℃ after reconstitution.Avoid repeated freezing and thawing. |
| 背景资料 | 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 7.5kb 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. |
| Uniprot ID | P54252 |
| 推荐配套试剂 | Biorab recommends Enhanced Chemiluminescent Kit with anti-Rabbit IgG (ZN1926) for Western blot,and HRP Conjugated anti-Rabbit IgG Super Vision Assay Kit (ZN1865) for IHC(P).*Blocking peptide可以联系我们购买。 |
| 基因名全称 | ataxin 3 |
| 蛋白名全称 | Ataxin-3 |
| 推荐稀释比 | Western blot(WB) 1:500～2000 Immunohistochemistry in paraffin section (IHC) 1:50～400 Immunocytochemistry/Immunofluorescence (ICC/IF) 1:50～400 Flow cytometry (FCM) 1～3μg/1×106 cells (Boiling the paraffin sections in 10mM citrate buffer,pH6.0,or PH8.0 EDTA repair liquid for 26 mins is required for the staining of formalin/paraffin sections.) Optimal working dilutions must be determined by end user. |
相关搜索：ATX3/ATXN3抗体，ATX3抗体，ATXN3多抗，Anti-ATX3/ATXN3 Antibody