Mitochondrial disorders are among the most frequent inborn errors of metabolism, their
primary cause being the dysfunction of the oxidative phosphorylation system (OXPHOS) …

Cytochrome c oxidase (COX) deficiency is characterized by a high degree of genetic and
phenotypic heterogeneity, partly reflecting the extreme structural complexity, multiple post …

Mitochondrial cytochrome c oxidase (CcO) or respiratory chain complex IV is a heme aa 3-
copper oxygen reductase containing metal centers essential for holo-complex biogenesis …

The mitochondrial proteome comprises~ 1000 (yeast)–1500 (human) different proteins,
which are distributed into four different subcompartments. The sublocalization of these …

The intermembrane space (IMS) represents the smallest subcompartment of mitochondria.
Nevertheless, it plays important roles in the transport and modification of proteins, lipids, and …

In eukaryotic cells, mitochondria perform cellular respiration through a series of redox
reactions ultimately reducing molecular oxygen to water. The system responsible for this …

Mitochondria are double-membrane organelles that contain their own genome, the
mitochondrial DNA (mtDNA), and reminiscent of its endosymbiotic origin. Mitochondria are …

Members of the coiled-coil-helix-coiled-coil-helix (CHCH) domain-containing protein family
that carry (CX 9 C) type motifs are imported into the mitochondrion with the help of the …

Twin CX9C proteins are eukaryotic proteins that derive their name from their characteristic
motif, consisting of two pairs of cysteines that form two disulfide bonds stabilizing a coiled …

The assembly of mitochondrial respiratory chain complex IV (cytochrome c oxidase) involves
the coordinated action of several assembly chaperones. In Saccharomyces cerevisiae, at …