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

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 …

Mitochondrial electron transport chain complexes are organized into supercomplexes
responsible for carrying out cellular respiration. Here we present three architectures of …

The assembly of the five oxidative phosphorylation system (OXPHOS) complexes in the
inner mitochondrial membrane is an intricate process. The human enzymes comprise core …

Mitochondrial complex I is the largest integral membrane enzyme of the respiratory chain
and consists of 44 different subunits encoded in the mitochondrial and nuclear genome. Its …

Mitochondria are composed of many small proteins that control protein synthesis, complex
assembly, metabolism, and ion and reactive oxygen species (ROS) handling. We show that …

The assembly of mitochondrial oxidative phosphorylation (OXPHOS) complexes is an
intricate process, which—given their dual-genetic control—requires tight co-regulation of two …

Primary mitochondrial diseases are relatively common inborn errors of energy metabolism,
with a combined prevalence of 1 in 4300. These disorders typically affect tissues with high …

Mitochondria are complex organelles whose dysfunction underlies a broad spectrum of
human diseases. Identifying all of the proteins resident in this organelle and understanding …