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 function depends on the correct synthesis, transport, and assembly of proteins
and cofactors of the electron transport chain. The initial idea that the respiratory chain …

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

Cytochrome c oxidase, the terminal enzyme of the respiratory chain, is assembled from
mitochondria-and nuclear-encoded subunits. The MITRAC complex represents the central …

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 …

Heme plays fundamental roles as cofactor and signaling molecule in multiple pathways
devoted to oxygen sensing and utilization in aerobic organisms. For cellular respiration …

Mitochondrial dysfunction is a significant factor in human disease, ranging from systemic
disorders of childhood to cardiomyopathy, ischaemia and neurodegeneration. Cytochrome …

Background We investigated a subject with an isolated cytochrome c oxidase (COX)
deficiency presenting with an unusual phenotype characterised by neuropathy, exercise …

Mitochondrial carrier homologs 1 (MTCH1) and 2 (MTCH2) are orphan members of the
mitochondrial transporter family SLC25. Human MTCH1 is also known as presenilin 1 …

The fruit fly—ie, Drosophila melanogaster—has proven to be a very useful model for the
understanding of basic physiological processes, such as development or ageing. The …