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DraconiticBiochemistryBiomolecules · Catabolism · Biosynthesis · MetabolismMap
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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

Outputs

Catalysed by

  • membrane insertase activity of COX18 [mitochondrial inner membrane]UniProt Q8N8Q8

Literature

  1. Human mitochondrial cytochrome c oxidase assembly factor COX18 acts transiently as a membrane insertase within the subunit 2 maturation module J Biol Chem, 2017
  2. 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
  3. Human COX20 cooperates with SCO1 and SCO2 to mature COX2 and promote the assembly of cytochrome c oxidase Hum Mol Genet, 2014
  4. The mitochondrial TMEM177 associates with COX20 during COX2 biogenesis Biochim Biophys Acta Mol Cell Res, 2018