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Target intelligence / Profile preview
Cytochrome c oxidase assembly factor 8 (COA8)
- Target
- COA8
- Molecular classification
- Other (mitochondrial assembly factor, non-enzymatic protein)
01
Overview
Cytochrome c oxidase assembly factor 8 (COA8) is a mitochondrial protein required for the assembly and stability of cytochrome c oxidase (COX, complex IV), the terminal enzyme in the respiratory electron transport chain. COA8 protects intermediate COX subunits from oxidative damage during assembly, particularly during states of increased cellular oxidative stress, thereby ensuring the integrity and function of mitochondrial energy generation. Mutations in COA8 result in complex IV deficiency and are associated with disorders such as mitochondrial leukoencephalopathy and peripheral neuropathy. Although formerly thought to play a role in apoptosis, current evidence confirms COA8's essential function is in COX assembly, not in programmed cell death.
- Other names
- Cytochrome c oxidase assembly factor 8COA8APOP1APOPT1C14orf153APOP-1MGC2562Apoptogenic protein 1, mitochondrialapoptogenic protein 1APOPMC4DN17UPF0671 protein C14orf153apoptogenic 1, mitochondrial
02
Mechanism of action
null
03
Biological functions
Assembly of cytochrome c oxidase (COX, complex IV of the mitochondrial respiratory chain)Protection of COX assembly from oxidation-induced degradationRegulation under oxidative stress conditions
04
Disease associations
Mitochondrial complex IV deficiencyNon-progressive predominantly posterior cavitating leukoencephalopathy with peripheral neuropathy
05
Safety considerations
Deficiency leads to multisystem mitochondrial disease, primarily affecting the central nervous system and muscle; dysfunction results in impaired energy metabolism and increased vulnerability to oxidative stress
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