人 ATXN3 抗体对
01AB03563
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.
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技术参数
- 靶点基因名
- ATXN3
- 基因别名
- ATX3; MJD; MJD1; SCA3
- 蛋白别名
- ataxin 3; ATXN3
- UniProt
- P54252
- 物种
- Human
产品详情
- 靶点名称
- ATXN3
- 蛋白名称
- ataxin 3
- 蛋白别名
- Ataxin-3; Machado-Joseph disease protein 1; Spinocerebellar ataxia type 3 protein
- 靶点基因名
- ATXN3
- 基因别名
- ATX3; MJD; MJD1; SCA3
- UniProt
- P54252
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
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.