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Target intelligence / Profile preview
Cytochrome c oxidase assembly factor 3 (COA3)
- Target
- COA3
- Molecular classification
- Other (assembly factor), Mitochondrial respiratory chain complex assembly factor
01
Overview
Cytochrome c oxidase assembly factor 3 (COA3) is a small inner mitochondrial membrane protein essential for the assembly of cytochrome c oxidase (complex IV) of the mitochondrial respiratory chain[1][2]. COA3 participates at early stages of complex IV assembly by stabilizing the core subunit COX1 and facilitating its integration with other structural subunits within the nascent complex. It functions within the MITRAC (mitochondrial translation regulation assembly intermediate of cytochrome c oxidase complex) complex and is vital for proper mitochondrial respiration and energy production. Mutations in COA3 cause complex IV deficiency with variable clinical phenotypes, mostly affecting muscle and nervous system tissues[1][2].
- Other names
- hCOA3MITRAC12CCDC56HSPC009COX25MC4DN14Coiled-coil domain-containing protein 56Mitochondrial translation regulation assembly intermediate of cytochrome c oxidase protein of 12 kDa
02
Biological functions
Mitochondrial respiratory chain assemblyCytochrome c oxidase (complex IV) biogenesisMitochondrial respirationProtein-protein interaction for enzyme assemblyRegulation of mitochondrial translation
03
Disease associations
Mitochondrial diseaseNeurodegenerative diseaseCardiomyopathyMyopathy
04
Safety considerations
Mutations can cause mitochondrial complex IV deficiency, leading to a broad spectrum of mitochondrial disorders including exercise intolerance, peripheral neuropathy, cardiomyopathy, liver dysfunction, and developmental delay
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