This is an old revision of this page, as edited by JCW-CleanerBot(talk | contribs) at 18:16, 4 September 2018(clean up, replaced: PloS One → PLOS One, removed stub tag). The present address (URL) is a permanent link to this revision, which may differ significantly from the current revision.
Revision as of 18:16, 4 September 2018 by JCW-CleanerBot(talk | contribs)(clean up, replaced: PloS One → PLOS One, removed stub tag)
Cytochrome c oxidase assembly factor 6 is a protein that in humans is encoded by the COA6gene.[5]Mitochondrial respiratory chainComplex IV, or cytochrome c oxidase, is the component of the respiratory chain that catalyzes the transfer of electrons from intermembrane spacecytochrome c to molecular oxygen in the matrix and as a consequence contributes to the proton gradient involved in mitochondrial ATP synthesis.[6][7] The COA6 gene encodes an assembly factor for mitochondrial complex IV and is a member of the cytochrome c oxidase subunit 6B family.[5][8] This protein is located in the intermembrane space, associating with SCO2 and COX2. It stabilizes newly formed COX2 and is part of the mitochondrial copper relay system.[9]Mutations in this gene result in fatal infantile cardioencephalomyopathy.[8]
Structure
The COA6 gene is located on the q arm of chromosome 1 in position 42.2 and spans 10,612 base pairs.[5] The gene produces a 14.1 kDa protein composed of 125 amino acids.[10][11] The COA6 protein is found a complex with TMEM177, COX20, MT-CO2/COX2, COX18, SCO1 and SCO2.[6][7] This protein localizes to the intermembrane space, associating with the inner membrane and transmembrane proteins such as SCO2 and COX2.[9]
Function
The COA6 encodes a protein which is an assembly factor for Complex IV.[5] This protein is specifically required for COX2 biogenesis and stability; the absence of this protein will cause fast turnover of newly synthesized COX2. As a constituent of mitochondrial copper relay system, this protein possibly relays copper ions from SCO2 to COX2.[9]
^ abcdPacheu-Grau D, Bareth B, Dudek J, Juris L, Vögtle FN, Wissel M, et al. (June 2015). "Cooperation between COA6 and SCO2 in COX2 maturation during cytochrome c oxidase assembly links two mitochondrial cardiomyopathies". Cell Metabolism. 21 (6): 823–33. doi:10.1016/j.cmet.2015.04.012. PMID25959673.