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 …

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

Iron is a crucial transition metal for virtually all life. Two major destinations of iron within
mammalian cells are the cytosolic iron-storage protein, ferritin, and mitochondria. In …

Mitochondria are essential for numerous cellular processes, yet hundreds of their proteins
lack robust functional annotation. To reveal functions for these proteins (termed MXPs), we …

Mitochondria play a key role in iron metabolism in that they synthesize heme, assemble iron–
sulfur (Fe/S) proteins, and participate in cellular iron regulation. Here, we review the latter …

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

Complex I (CI) is the largest protein complex in the mitochondrial oxidative phosphorylation
electron transport chain of the inner mitochondrial membrane and plays a key role in the …

The mitochondrion is arguably the most complex organelle in the budding yeast cell
cytoplasm. It is essential for viability as well as respiratory growth. Its innermost aqueous …

Electron transport chain (ETC) biogenesis is tightly coupled to energy levels and availability
of ETC subunits. Complex III (CIII), controlling ubiquinol: ubiquinone ratio in ETC, is an …