SCO Cytochrome Oxidase Deficient Homolog 2, commonly referred to as SCO2, is a protein encoded by the SCO2 gene in humans. This protein plays a crucial role in the assembly and function of cytochrome c oxidase (COX), which is a key component of the mitochondrial respiratory chain. The recombinant form of this protein is produced using various expression systems for research and therapeutic purposes.
The SCO2 gene is located on the q arm of chromosome 22 at position 13.33 and spans approximately 2,871 base pairs . The gene produces a 15.1 kDa protein composed of 136 amino acids . The protein contains an N-terminal mitochondrial targeting presequence of 41 amino acids, which is essential for its localization to the mitochondria .
SCO2 is a metallochaperone involved in the biogenesis of cytochrome c oxidase subunit II (MT-CO2/COX2). It is essential for the synthesis and maturation of this subunit by transporting copper to the Cu(A) site on MT-CO2/COX2 . Additionally, SCO2 acts as a thiol-disulfide oxidoreductase, regulating the redox state of cysteines in SCO1 during the maturation of MT-CO2/COX2 .
Cytochrome c oxidase (COX) catalyzes the transfer of electrons from cytochrome c to molecular oxygen, which helps maintain the proton gradient across the inner mitochondrial membrane. This gradient is necessary for aerobic ATP production . Therefore, SCO2 is vital for cellular energy production and overall mitochondrial function.
The recombinant form of SCO2 is produced using expression systems such as E. coli. This recombinant protein is used in various research applications to study its function, interactions, and role in diseases . The recombinant SCO2 protein is typically purified to high levels of purity and is available in lyophilized form for ease of use in laboratory settings .