We showed that the human respiratory chain is organized in supramolecular assemblies of
respiratory chain complexes, the respirasomes. The mitochondrial complexes I (NADH
dehydrogenase) and III (cytochrome c reductase) form a stable core respirasome to which
complex IV (cytochrome c oxidase) can also bind. An analysis of the state of respirasomes in
patients with an isolated deficiency of single complexes provided evidence that the
formation of respirasomes is essential for the assembly/stability of complex I, the major entry …