人 CHMP2B 抗体对
01AB01064
Charged multivesicular body protein 2B (CHMP2B) is a component of the ESCRT-III complex, which is crucial for endosomal sorting and multivesicular body formation. The protein is encoded by the CHMP2B gene on chromosome 3 and is primarily active in the brain. CHMP2B forms long chains that spiral around budding vesicles, constricting their necks before cleavage from the membrane. It contains a C-terminal domain that regulates its activity and interaction with other ESCRT-III subunits. CHMP2B is essential for neuronal survival and proper protein degradation through the endosomal-lysosomal pathway. Mutations in CHMP2B, particularly those affecting the C-terminal domain, are associated with chromosome 3-linked frontotemporal dementia (FTD3). These mutations lead to the production of abnormal CHMP2B proteins that disrupt protein transport and degradation, ultimately causing neuronal death in the frontal and temporal lobes of the brain.
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技术参数
- 靶点基因名
- CHMP2B
- 蛋白别名
- charged multivesicular body protein 2B; CHMP2B
- UniProt
- Q9UQN3
- 物种
- Human
产品详情
- 靶点名称
- CHMP2B
- 蛋白名称
- charged multivesicular body protein 2B
- 蛋白别名
- Charged multivesicular body protein 2b; CHMP2.5; Chromatin-modifying protein 2b; CHMP2b; Vacuolar protein sorting-associated protein 2-2; Vps2-2; hVps2-2
- 靶点基因名
- CHMP2B
- UniProt
- Q9UQN3
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
Charged multivesicular body protein 2B (CHMP2B) is a component of the ESCRT-III complex, which is crucial for endosomal sorting and multivesicular body formation. The protein is encoded by the CHMP2B gene on chromosome 3 and is primarily active in the brain. CHMP2B forms long chains that spiral around budding vesicles, constricting their necks before cleavage from the membrane. It contains a C-terminal domain that regulates its activity and interaction with other ESCRT-III subunits. CHMP2B is essential for neuronal survival and proper protein degradation through the endosomal-lysosomal pathway. Mutations in CHMP2B, particularly those affecting the C-terminal domain, are associated with chromosome 3-linked frontotemporal dementia (FTD3). These mutations lead to the production of abnormal CHMP2B proteins that disrupt protein transport and degradation, ultimately causing neuronal death in the frontal and temporal lobes of the brain.