COX18 inserts nascent MT-CO2 in COX20:TMEM77
Mg2+ + COX20:TMEM177 + nascent MT-CO2 → MT-CO2:COX20:TMEM177
- Reactome
- R-HSA-9902096
- Compartment
- mitochondrial inner membrane · mitochondrial matrix
- Rhea
- None recorded
Inputs
- Mg2+CHEBI:18420, Mg, mitochondrial matrix
- COX20:TMEM177R-HSA-9865587, mitochondrial inner membrane
- nascent MT-CO2R-HSA-9902095, mitochondrial matrix
Outputs
- MT-CO2:COX20:TMEM177R-HSA-9865557, mitochondrial inner membrane
Catalysed by
- membrane insertase activity of COX18 [mitochondrial inner membrane]UniProt Q8N8Q8
Literature
- Human mitochondrial cytochrome c oxidase assembly factor COX18 acts transiently as a membrane insertase within the subunit 2 maturation module J Biol Chem, 2017
- A mutation in the FAM36A gene, the human ortholog of COX20, impairs cytochrome c oxidase assembly and is associated with ataxia and muscle hypotonia Hum Mol Genet, 2013
- Human COX20 cooperates with SCO1 and SCO2 to mature COX2 and promote the assembly of cytochrome c oxidase Hum Mol Genet, 2014
- The mitochondrial TMEM177 associates with COX20 during COX2 biogenesis Biochim Biophys Acta Mol Cell Res, 2018