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

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

The chapter provides a review of the state of art of the oxidative phosphorylation system in
mammalian mitochondria. The sections of the paper deal with:(i) the respiratory chain as a …

The enzymatic complexes of the mitochondrial respiratory chain have been extensively
investigated in their structural and functional properties. A clear distinction is possible today …

All known eukaryotes require copper for their development and survival. The essentiality of
copper reflects its widespread use as a co-factor in conserved enzymes that catalyze …

Metals are essential elements of all living organisms. Among them, copper is required for a
multiplicity of functions including mitochondrial oxidative phosphorylation and protection …

The main cellular source of reactive oxygen species (ROS) is mitochondrial respiratory
chain and active NADPH responsible for “respiratory burst” of phagocytes. Whatsmore ROS …

Assembly of the oxidative phosphorylation (OXPHOS) system in the mitochondrial inner
membrane is an intricate process in which many factors must interact. The OXPHOS system …

Mitochondrial ATPases associated with diverse cellular activities (AAA) proteases are
involved in the quality control and processing of inner-membrane proteins. Here we …

The biogenesis of human cytochrome c oxidase (COX) is an intricate process in which three
mitochondrial DNA (mtDNA)-encoded core subunits are assembled in a coordinated way …