人 TACO1 抗体对
01AB01605
Translational activator of cytochrome c oxidase I (TACO1) is a mitochondrial protein that plays a crucial role in the translation of the mitochondrially encoded COX1 (cytochrome c oxidase subunit I) mRNA, which is essential for the assembly and function of cytochrome c oxidase, the terminal enzyme of the mitochondrial respiratory chain. Structurally, TACO1 consists of three domains forming a hook-like shape, with domain 1 as a helical bundle and domains 2 and 3 as αβ-sandwiches; these domains interact via hydrophobic and polar contacts, and a large tunnel separates domains 1 and 3, contributing to the protein’s stability and function. TACO1 binds to the mitoribosome and facilitates translation elongation, preventing premature termination and ensuring proper synthesis of full-length COX1 protein. Mutations in TACO1 disrupt this process, leading to cytochrome c oxidase deficiency and are associated with late-onset Leigh syndrome, a severe neurodegenerative disorder characterized by progressive loss of mental and movement abilities.
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技术参数
- 靶点基因名
- TACO1
- 基因别名
- CCDC44
- 蛋白别名
- translational activator of cytochrome c oxidase I; TACO1
- UniProt
- Q9BSH4
- 物种
- Human
产品详情
- 靶点名称
- TACO1
- 蛋白名称
- translational activator of cytochrome c oxidase I
- 蛋白别名
- Translational activator of cytochrome c oxidase 1; Coiled-coil domain-containing protein 44; Translational activator of mitochondrially-encoded cytochrome c oxidase I
- 靶点基因名
- TACO1
- 基因别名
- CCDC44
- UniProt
- Q9BSH4
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
Translational activator of cytochrome c oxidase I (TACO1) is a mitochondrial protein that plays a crucial role in the translation of the mitochondrially encoded COX1 (cytochrome c oxidase subunit I) mRNA, which is essential for the assembly and function of cytochrome c oxidase, the terminal enzyme of the mitochondrial respiratory chain. Structurally, TACO1 consists of three domains forming a hook-like shape, with domain 1 as a helical bundle and domains 2 and 3 as αβ-sandwiches; these domains interact via hydrophobic and polar contacts, and a large tunnel separates domains 1 and 3, contributing to the protein’s stability and function. TACO1 binds to the mitoribosome and facilitates translation elongation, preventing premature termination and ensuring proper synthesis of full-length COX1 protein. Mutations in TACO1 disrupt this process, leading to cytochrome c oxidase deficiency and are associated with late-onset Leigh syndrome, a severe neurodegenerative disorder characterized by progressive loss of mental and movement abilities.