ARG56261
anti-Ataxin 3 antibody
anti-Ataxin 3 antibody for ICC/IF,IHC-Formalin-fixed paraffin-embedded sections,Western blot and Human,Mouse,Rat
概述
| 产品描述 | Rabbit Polyclonal antibody recognizes Ataxin 3 |
|---|---|
| 反应物种 | Hu, Ms, Rat |
| 应用 | ICC/IF, IHC-P, WB |
| 宿主 | Rabbit |
| 克隆 | Polyclonal |
| 同位型 | IgG |
| 靶点名称 | Ataxin 3 |
| 抗原物种 | Human |
| 抗原 | Recombinant protein of Human Ataxin-3 |
| 偶联标记 | Un-conjugated |
| 別名 | MJD; ATX3; JOS; Spinocerebellar ataxia type 3 protein; Ataxin-3; Machado-Joseph disease protein 1; AT3; SCA3; MJD1; EC 3.4.19.12 |
应用说明
| 应用建议 |
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| 应用说明 | * The dilutions indicate recommended starting dilutions and the optimal dilutions or concentrations should be determined by the scientist. | ||||||||
| 阳性对照 | SW480 |
属性
| 形式 | Liquid |
|---|---|
| 纯化 | Affinity purification with immunogen. |
| 缓冲液 | PBS (pH 7.3), 0.02% Sodium azide and 50% Glycerol. |
| 抗菌剂 | 0.02% Sodium azide |
| 稳定剂 | 50% Glycerol |
| 存放说明 | For continuous use, store undiluted antibody at 2-8°C for up to a week. For long-term storage, aliquot and store at -20°C. Storage in frost free freezers is not recommended. Avoid repeated freeze/thaw cycles. Suggest spin the vial prior to opening. The antibody solution should be gently mixed before use. |
| 注意事项 | For laboratory research only, not for drug, diagnostic or other use. |
生物信息
| 数据库连接 | |
|---|---|
| 基因名称 | ATXN3 |
| 全名 | ataxin 3 |
| 背景介绍 | 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 13-36 to 68-79 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. [provided by RefSeq, Sep 2009] |
| 生物功能 | Deubiquitinating enzyme involved in protein homeostasis maintenance, transcription, cytoskeleton regulation, myogenesis and degradation of misfolded chaperone substrates. Binds long polyubiquitin chains and trims them, while it has weak or no activity against chains of 4 or less ubiquitins. Involved in degradation of misfolded chaperone substrates via its interaction with STUB1/CHIP: recruited to monoubiquitinated STUB1/CHIP, and restricts the length of ubiquitin chain attached to STUB1/CHIP substrates and preventing further chain extension. In response to misfolded substrate ubiquitination, mediates deubiquitination of monoubiquitinated STUB1/CHIP. Interacts with key regulators of transcription and represses transcription: acts as a histone-binding protein that regulates transcription. [UniProt] |
| 预测分子量 | 41 kDa |
| 翻译后修饰 | Monoubiquitinated N-terminally by UBE2W, possibly leading to activate the deubiquitinating enzyme activity. |
检测图片 (3) Click the Picture to Zoom In
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ARG56261 anti-Ataxin 3 antibody ICC/IF image
Immunofluorescence: HeLa cells stained with ARG56261 anti-Ataxin 3 antibody.
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ARG56261 anti-Ataxin 3 antibody IHC-P image
Immunohistochemistry: Formalin/PFA-fixed paraffin-embedded Rat kidney tissue stained with ARG56261 anti-Ataxin 3 antibody at 1:200 dilution.
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ARG56261 anti-Ataxin 3 antibody WB image
Western blot: SW480 cell lysate stained with ARG56261 anti-Ataxin 3 antibody.
