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

Mitochondria are double‐membrane‐bound organelles that are present in all nucleated
eukaryotic cells and are responsible for the production of cellular energy in the form of ATP …

Mitochondria are ubiquitous intracellular organelles found in almost all eukaryotes and
involved in various aspects of cellular life, with a primary role in energy production. The …

Cardiac tissue requires a persistent production of energy in order to exert its pum**
function. Therefore, the maintenance of this function relies on mitochondria that represent …

Human complex II is a key protein complex that links two essential energy-producing
processes: the tricarboxylic acid cycle and oxidative phosphorylation. Deficiencies due to …

Increasing evidence points to the respiratory Complex II (CII) as a source and modulator of
reactive oxygen species (ROS). Both functional loss of CII as well as its pharmacological …

Abstract In October 2016, the American Association for Cancer Research held a meeting of
international childhood cancer predisposition syndrome experts to evaluate the current …

Succinate dehydrogenase (SDH) also known as complex II or succinate: quinone
oxidoreductase is an enzyme involved in both oxidative phosphorylation and tricarboxylic …

Here we report whole exome sequencing (WES) on a cohort of 71 patients with persistently
unresolved white matter abnormalities with a suspected diagnosis of leukodystrophy or …

Loss of function of the succinate dehydrogenase complex characterizes a rare group of
human tumors including some gastrointestinal stromal tumors, paragangliomas, renal …