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

Driver mutations in oncogenic pathways, rewiring of cellular metabolism and altered ROS
homoeostasis are intimately connected hallmarks of cancer. Electrons derived from different …

Mitochondrially generated reactive oxygen species are involved in a myriad of signaling and
damaging pathways in different tissues. In addition, mitochondria are an important target of …

The current state of knowledge on how copper metallochaperones support the maturation of
cuproproteins is reviewed. Copper is needed within mitochondria to supply the CuA and …

Mitochondria are essential organelles of endosymbiotic origin that are responsible for
oxidative phosphorylation within eukaryotic cells. Independent evolution between species …

Cytochrome c oxidase (CcO) is the terminal oxidase of the mitochondrial electron transport
chain. This bigenomic enzyme in mammals contains 13 subunits of which the 3 catalytic …

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

All living organisms require nutrient minerals for growth and have developed mechanisms to
acquire, utilize, and store nutrient minerals effectively. In the aqueous cellular environment …

The sequential flow of electrons in the respiratory chain, from a low reduction potential
substrate to O2, is mediated by protein-bound redox cofactors. In mitochondria, hemes …

Mitochondrial respiratory-chain complexes assemble from subunits of dual genetic origin
assisted by specialized assembly factors. Whereas core subunits are translated on …