人 TIMM8B 抗体对
01AB02106
Translocase of inner mitochondrial membrane 8 homolog B (TIMM8B) is a highly conserved mitochondrial protein that functions as a probable chaperone within the mitochondrial intermembrane space, where it forms heterooligomeric complexes essential for the import and insertion of multi-pass transmembrane proteins into the inner mitochondrial membrane. TIMM8B is unstable as a monomer but gains stability in hexameric complexes, often partnering with other small TIM proteins to facilitate the assembly of mitochondrial respiratory chain complexes, particularly impacting the abundance and assembly of Complex IV subunits and associated copper chaperones. Loss or dysfunction of TIMM8B disrupts the assembly of these complexes, leading to increased reactive oxygen species and mitochondrial dysfunction, which has been associated with cellular sensitivity to oxidative stress and may contribute to mitochondrial disease phenotypes.
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技术参数
- 靶点基因名
- TIMM8B
- 基因别名
- DDP2; DDPL; TIM8B
- 蛋白别名
- translocase of inner mitochondrial membrane 8 homolog B; TIMM8B
- UniProt
- Q9Y5J9
- 物种
- Human
产品详情
- 靶点名称
- TIMM8B
- 蛋白名称
- translocase of inner mitochondrial membrane 8 homolog B
- 蛋白别名
- Mitochondrial import inner membrane translocase subunit Tim8 B; DDP-like protein; Deafness dystonia protein 2
- 靶点基因名
- TIMM8B
- 基因别名
- DDP2; DDPL; TIM8B
- UniProt
- Q9Y5J9
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
Translocase of inner mitochondrial membrane 8 homolog B (TIMM8B) is a highly conserved mitochondrial protein that functions as a probable chaperone within the mitochondrial intermembrane space, where it forms heterooligomeric complexes essential for the import and insertion of multi-pass transmembrane proteins into the inner mitochondrial membrane. TIMM8B is unstable as a monomer but gains stability in hexameric complexes, often partnering with other small TIM proteins to facilitate the assembly of mitochondrial respiratory chain complexes, particularly impacting the abundance and assembly of Complex IV subunits and associated copper chaperones. Loss or dysfunction of TIMM8B disrupts the assembly of these complexes, leading to increased reactive oxygen species and mitochondrial dysfunction, which has been associated with cellular sensitivity to oxidative stress and may contribute to mitochondrial disease phenotypes.