Anti-Human COA4 Antibody Pair

Product Description: 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. Protein Function: Putative COX assembly factor [The Uniprot Consortium]