The reconstitution of a respiratory chain1 may be defined as the reassembly of the components to form an integral multienzyme system identical with the original. In the case of the succinate oxidase system, for example, the reconstituted particle shows all the properties of the original preparation as described in some of the papers of this series (e.g.(2-4)). This paper describes recent eviden...

Oxidative phosphorylation, i.e., ATP synthesis by the oxygen-consuming respiratory chain (RC), supplies most organs and tissues with a readily usable energy source, being functional before birth. Consequently, RC deficiencies can theoretically give rise to any symptom, in any organ or tissue, at any age and with any mode of inheritance, because of the twofold genetic origin of RC components (nu...

The reconstitution of a respiratory chain1 may be defined as the reassembly of the components to form an integral multienzyme system identical with the original. In the case of the succinate oxidase system, for example, the reconstituted particle shows all the properties of the original preparation as described in some of the papers of this series (e.g.(2-4)). This paper describes recent eviden...

OBJECTIVE In this study, we report 5 patients with heterogeneous phenotypes and biochemical evidence of respiratory chain (RC) deficiency; however, the molecular diagnosis is not mitochondrial disease. METHODS The reported patients were identified from a cohort of 60 patients in whom RC enzyme deficiency suggested mitochondrial disease and underwent whole-exome sequencing. RESULTS Five pati...

Cell and tissue damage in respiratory chain disorders have been related to increased production of reactive oxygen species (ROS). We measured telomere lengths in such disorders since ROS have also been implicated with telomere shortening. We investigated whole blood cell DNA of 14 patients with MELAS-related mitochondriopathy and two patients with the LHON-associated G11778A mutation of the mit...

Mitochondrial Respiratory Chain Disorders (MRCD) are a heterogeneous group of disorders that share the involvement of the cellular bioenergetic machinery due to molecular defects affecting the mitochondrial oxidative phosphorylation system (OXPHOS). Clinically, they usually involve multiple tissues although they tend to mainly affect nervous system and skeletal muscle. Cardiological manifestati...

For decades mitochondria have been considered static round-shaped organelles in charge of energy production. In contrast, they are highly dynamic cellular components that undergo continuous cycles of fusion and fission influenced, for instance, by oxidative stress, cellular energy requirements, or the cell cycle state. New important functions beyond energy production have been attributed to mit...

Keilin's classic paper of 1925 [Keilin (1925) Proc. R. Soc. London Ser. B 100, 129-151], achieved with simple, but elegant, techniques, describes the cytochrome components of the respiratory chain and their roles in intracellular respiration and oxygen consumption. Since that time, a tremendous amount of work has clarified the intricate details of the prosthetic groups, cofactors and proteins t...

The respiratory chain and oxidative phosphorylation are of utter importance for a cell’s metabolism. Here, the energy won from ingested food is being converted into our body’s energy unit (adenosine triphosphate). As the name respiratory chain suggests, oxygen also plays an important role. The following article will explain how exactly this complex mechanism works and which substances may disab...