Anti-Human TACO1 Antibody Pair

Product Description: 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 alphabeta-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. Protein Function: Acts as a translational activator of mitochondrially-encoded cytochrome c oxidase 1 [The Uniprot Consortium]