Ataxin 3 抗体
Quick Overview for Ataxin 3 抗体 (ABIN2666310)
抗原
See all Ataxin 3 (ATXN3) 抗体适用
宿主
克隆类型
标记
应用范围
克隆位点
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纯化方法
- The antibody was purified by affinity chromatography.
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亚型
- IgG1 kappa
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应用备注
- Optimal working dilution should be determined by the investigator.
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限制
- 仅限研究用
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浓度
- 0.5 mg/mL
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缓冲液
- phosphate-buffered solution, pH 7.2, containing 0.05 % sodium azide.
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储存液
- Sodium azide
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注意事项
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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储存条件
- 4 °C
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储存方法
- Upon receipt, store undiluted between 2°C and 8°C.
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- Ataxin 3 (ATXN3)
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别名
- Ataxin-3
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背景
- Ataxin-3 is an ubiquitously expressed protein which contains a poly-glutamine tract. Genetically abnormal expansion of the poly-glutamine repeats results in accumulation of ubiquitinated ataxin-3 protein aggregates and leads to a neurodegenerative disorder, Machado-Joseph disease (MJD). The protein has ubiquitin interacting motifs and a N-terminal Cysteine residue (C14) responsible for its deubiquitinating activity. Ataxin-3 binds and hydrolyzes polyubiquitinated proteins, and interacts with key proteasome components, such as p97/VCP and p45, indicating that Ataxin-3 may play functional roles in ubiquitin-proteasome system. The protein also act as a transcriptional repressor when interacting with transcriptional activators and coactivators.
抗原
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