Mitochondrial COA7 is a heme-binding protein with disulfide reductase activity, which acts in the early stages of complex IV assembly
LE Formosa, S Maghool, AJ Sharpe… - Proceedings of the …, 2022 - National Acad Sciences
Cytochrome c oxidase (COX) assembly factor 7 (COA7) is a metazoan-specific assembly
factor, critical for the biogenesis of mitochondrial complex IV (cytochrome c oxidase) …
factor, critical for the biogenesis of mitochondrial complex IV (cytochrome c oxidase) …
Structural and functional characterization of the mitochondrial complex IV assembly factor Coa6
Assembly factors play key roles in the biogenesis of many multi-subunit protein complexes
regulating their stability, activity, and the incorporation of essential cofactors. The human …
regulating their stability, activity, and the incorporation of essential cofactors. The human …
[HTML][HTML] COA6 facilitates cytochrome c oxidase biogenesis as thiol-reductase for copper metallochaperones in mitochondria
D Pacheu-Grau, M Wasilewski, S Oeljeklaus… - Journal of molecular …, 2020 - Elsevier
The mitochondrial cytochrome c oxidase, the terminal enzyme of the respiratory chain,
contains heme and copper centers for electron transfer. The conserved COX2 subunit …
contains heme and copper centers for electron transfer. The conserved COX2 subunit …
hCOA3 stabilizes cytochrome c oxidase 1 (COX1) and promotes cytochrome c oxidase assembly in human mitochondria
P Clemente, S Peralta, A Cruz-Bermudez… - Journal of Biological …, 2013 - ASBMB
Cytochrome c oxidase (COX) or complex IV of the mitochondrial respiratory chain plays a
fundamental role in energy production of aerobic cells. In humans, COX deficiency is the …
fundamental role in energy production of aerobic cells. In humans, COX deficiency is the …
COA6 is structurally tuned to function as a thiol-disulfide oxidoreductase in copper delivery to mitochondrial cytochrome c oxidase
In eukaryotes, cellular respiration is driven by mitochondrial cytochrome c oxidase (CcO), an
enzyme complex that requires copper cofactors for its catalytic activity. Insertion of copper …
enzyme complex that requires copper cofactors for its catalytic activity. Insertion of copper …
Copper supplementation restores cytochrome c oxidase assembly defect in a mitochondrial disease model of COA6 deficiency
A Ghosh, PP Trivedi, SA Timbalia… - Human molecular …, 2014 - academic.oup.com
Mitochondrial respiratory chain biogenesis is orchestrated by hundreds of assembly factors,
many of which are yet to be discovered. Using an integrative approach based on clues from …
many of which are yet to be discovered. Using an integrative approach based on clues from …
A mutation in the FAM36A gene, the human ortholog of COX20, impairs cytochrome c oxidase assembly and is associated with ataxia and muscle hypotonia
R Szklarczyk, BFJ Wanschers… - Human molecular …, 2013 - academic.oup.com
The mitochondrial respiratory chain complex IV (cytochrome c oxidase) is a multi-subunit
enzyme that transfers electrons from cytochrome c to molecular oxygen, yielding water. Its …
enzyme that transfers electrons from cytochrome c to molecular oxygen, yielding water. Its …
What Role Does COA6 Play in Cytochrome C Oxidase Biogenesis: A Metallochaperone or Thiol Oxidoreductase, or Both?
Complex IV (cytochrome c oxidase; COX) is the terminal complex of the mitochondrial
electron transport chain. Copper is essential for COX assembly, activity, and stability, and is …
electron transport chain. Copper is essential for COX assembly, activity, and stability, and is …
Human mitochondrial cytochrome c oxidase assembly factor COX18 acts transiently as a membrane insertase within the subunit 2 maturation module
M Bourens, A Barrientos - Journal of Biological Chemistry, 2017 - ASBMB
Defects in mitochondrial cytochrome c oxidase or respiratory chain complex IV (CIV)
assembly are a frequent cause of human mitochondrial disorders. Specifically, mutations in …
assembly are a frequent cause of human mitochondrial disorders. Specifically, mutations in …
Mitochondrial disease genes COA6, COX6B and SCO2 have overlapping roles in COX2 biogenesis
A Ghosh, AT Pratt, S Soma, SG Theriault… - Human molecular …, 2016 - academic.oup.com
Biogenesis of cytochrome c oxidase (CcO), the terminal enzyme of the mitochondrial
respiratory chain, is a complex process facilitated by several assembly factors. Pathogenic …
respiratory chain, is a complex process facilitated by several assembly factors. Pathogenic …