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

Complex I (NADH: ubiquinone oxidoreductase) is the largest multimeric enzyme complex of
the mitochondrial respiratory chain, which is responsible for electron transport and the …

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 …

Aims: The mitochondrial respiratory chain is recognized today to be arranged in
supramolecular assemblies (supercomplexes). Besides conferring a kinetic advantage …

Cytoplasmic RNA granules play a central role in mRNA metabolism, but the importance of
mitochondrial RNA granules remains relatively unexplored. We characterized their …

α-Synuclein, a protein implicated in the pathogenesis of Parkinson disease (PD), is thought
to affect mitochondrial functions, although the mechanisms of its action remain unclear. In …

Loss of mitochondrial complex I catalytic activity in the electron transport chain (ETC) is
found in multiple tissues from individuals with sporadic Parkinson's disease (PD) and is a …

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 …

Mitochondria are ubiquitous organelles in eukaryotic cells whose primary function is to
generate energy supplies in the form of ATP through oxidative phosphorylation. As the entry …

A puzzling observation in patients with oxidative phosphorylation (OXPHOS) deficiencies is
the presence of combined enzyme complex defects associated with a genetic alteration in …