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Target intelligence / Profile preview
Cytochrome c oxidase assembly factor 1 homolog (COA1)
- Target
- COA1
- Molecular classification
- Other (mitochondrial assembly factor)
01
Overview
Cytochrome c oxidase assembly factor 1 homolog (COA1) is a mitochondrial inner membrane protein required for the proper assembly of cytochrome c oxidase (complex IV) of the respiratory chain. COA1 interacts with other assembly factors involved in mitochondrial translation and complex IV biogenesis (such as Shy1/SURF1, Mss51, and Cox14) and is essential for the formation and stabilization of assembly intermediates required to produce mature and functional cytochrome c oxidase. Loss of COA1 activity impairs complex IV biogenesis, leading to deficiency of this crucial respiratory chain complex and causing various mitochondrial pathologies, including severe disorders such as Leigh syndrome.
- Other names
- COA1C7orf44MITRAC15FLJ10803Mitochondrial translation regulation assembly intermediate of cytochrome c oxidase protein of 15 kDacytochrome c oxidase assembly factor 1 homologcytochrome c oxidase assembly protein 1 homolog
02
Biological functions
Mitochondrial respiratory chain complex IV (cytochrome c oxidase) assemblyRegulation of mitochondrial translation and biogenesisProtein–protein interaction within mitochondrial inner membrane
03
Disease associations
Mitochondrial disorders (e.g., Leigh syndrome, via impaired complex IV assembly)Other mitochondrial dysfunctions
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