抗人 COA4 抗体对
01AB00781
Cytochrome c oxidase assembly factor 4 homolog (COA4) is a small protein involved in the assembly of cytochrome c oxidase, the terminal enzyme of the mitochondrial respiratory chain. COA4 is localized in the mitochondrial intermembrane space and plays a crucial role in the biogenesis and stability of cytochrome c oxidase. It contains a conserved twin CX9C motif that forms two intramolecular disulfide bonds, contributing to its structural stability. COA4 interacts with other assembly factors and subunits of cytochrome c oxidase, facilitating the proper assembly of the enzyme complex. Mutations in the COA4 gene have been associated with mitochondrial complex IV deficiency, a rare genetic disorder characterized by reduced cytochrome c oxidase activity. This condition can lead to various clinical manifestations, including muscle weakness, developmental delay, and neurological problems.
| 规格 | 起售价 |
|---|---|
| 登录后可查看 | |
技术参数
- 靶点基因名
- COA4
- 基因别名
- CHCHD8; E2IG2
- 蛋白别名
- cytochrome c oxidase assembly factor 4 homolog; COA4
- UniProt
- Q9NYJ1
- 物种
- Human
产品详情
- 靶点名称
- COA4
- 蛋白名称
- cytochrome c oxidase assembly factor 4 homolog
- 蛋白别名
- Cytochrome c oxidase assembly factor 4 homolog; mitochondrial; Coiled-coil-helix-coiled-coil-helix domain-containing protein 8; E2-induced gene 2 protein
- 靶点基因名
- COA4
- 基因别名
- CHCHD8; E2IG2
- UniProt
- Q9NYJ1
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
Cytochrome c oxidase assembly factor 4 homolog (COA4) is a small protein involved in the assembly of cytochrome c oxidase, the terminal enzyme of the mitochondrial respiratory chain. COA4 is localized in the mitochondrial intermembrane space and plays a crucial role in the biogenesis and stability of cytochrome c oxidase. It contains a conserved twin CX9C motif that forms two intramolecular disulfide bonds, contributing to its structural stability. COA4 interacts with other assembly factors and subunits of cytochrome c oxidase, facilitating the proper assembly of the enzyme complex. Mutations in the COA4 gene have been associated with mitochondrial complex IV deficiency, a rare genetic disorder characterized by reduced cytochrome c oxidase activity. This condition can lead to various clinical manifestations, including muscle weakness, developmental delay, and neurological problems.