Overview
| 别名 | Ataxin-3; Machado-Joseph disease protein 1; Spinocerebellar ataxia type 3 protein |
| 基因名 | ATXN3 |
| UniProt ID | P54252 |
| 反应种属 | Human |
| 应用 | WB,IHC-P |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG1 |
| 克隆号 | 1S2-U7-R3 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 41 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | WB-1:1000; IHC-1:100-1:200 |
| 存储缓冲液 | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.09% sodium azide |
| 保存温度 | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| 背景信息 | Ataxin 3 (ATXN3) is a ubiquitously expressed deubiquitinating (DUB) enzyme that plays a critical role in proteostasis, DNA repair, and transcriptional regulation. Structurally, it features an N-terminal Josephin domain with catalytic activity, tandem ubiquitin-interacting motifs, and a polyglutamine repeat region. As a chain-editing enzyme, it preferentially cleaves K63-linked polyubiquitin chains and collaborates with the p97 ATPase in endoplasmic reticulum-associated degradation. It also interacts with autophagy proteins such as LC3C and GABARAP to facilitate the retrograde transport of misfolded proteins to aggresomes. In the nucleus, ATXN3 assists in DNA repair by interacting with PNKP and regulating the checkpoint kinase Chk1. Expansion of the polyglutamine region within the protein leads to Spinocerebellar Ataxia Type 3 (SCA3), a neurodegenerative disorder characterized by protein aggregation and the disruption of cellular processes in neuronal tissue. |
检测原理