Cytochrome bd is a respiratory quinol: O2 oxidoreductase found in many prokaryotes,
including a number of pathogens. The main bioenergetic function of the enzyme is the …

Coenzyme Q (CoQ, ubiquinone) is the electron‐carrying lipid in the mitochondrial electron
transport system (ETS). In mammals, it serves as the electron acceptor for nine mitochondrial …

For the vast majority of patients with mitochondrial diseases, only supportive and
symptomatic therapies are available. However, in the last decade, due to extraordinary …

As with other mitochondrial respiratory chain components, marked clinical and genetic
heterogeneity is observed in patients with a cytochrome c oxidase deficiency. This …

Primary mitochondrial diseases represent some of the most common and severe inherited
metabolic disorders, affecting~ 1 in 4,300 live births. The clinical and molecular diversity …

The transcription factor hypoxia-inducible factor-1α (HIF-1α) is a master regulator of the
cellular response to low oxygen. HIF-1α protein accumulates in hypoxia due to inhibition of …

Based on studies with a fluorescent reporter dye, Mito Thermo Yellow (MTY), and the
genetically encoded gTEMP ratiometric fluorescent temperature indicator targeted to …

The mitochondria have arisen as a consequence of endosymbiosis of an ancestral α-
proteobacterium with a methane-producing archae. The main function of the canonical …

Mitochondrial diseases are extremely heterogeneous genetic conditions characterized by
faulty oxidative phosphorylation (OXPHOS). OXPHOS deficiency can be the result of …

Mitochondrial disorders are an important group of genetic conditions characterized by
impaired oxidative phosphorylation. Mitochondrial disorders come with an impressive …