Protein SCO1 homolog, mitochondrial (SCO1)

Protein SCO1 homolog, mitochondrial (SCO1) is a copper metallochaperone essential for the assembly and maturation of cytochrome c oxidase subunit II (COX2 or MT-CO2) in the mitochondrial respiratory chain. It is embedded in the inner mitochondrial membrane, where it facilitates copper delivery to the enzyme complex required for efficient electron transfer and ATP synthesis via oxidative phosphorylation. SCO1 is crucial for copper homeostasis and redox balance in cells with high metabolic demand. Mutations in SCO1 result in tissue-specific mitochondrial complex IV deficiencies manifesting as severe, often fatal, multisystem disorders including hepatic failure, cardiomyopathy, and encephalopathy. SCO1 interacts directly with other COX assembly factors and is part of a conserved mitochondrial copper delivery pathway

Other names

SCO1Synthesis of cytochrome c oxidase 1SCO1, cytochrome c oxidase assembly proteinSCOD1MC4DN4SCO cytochrome c oxidase assembly protein 1SCO cytochrome oxidase deficient homolog 1

Molecules that target or modulate SCO1 would likely affect the assembly/stability of cytochrome c oxidase, copper delivery, or mitochondrial electron transport