The mitochondrial oxidative phosphorylation system is central to cellular metabolism. It
comprises five enzymatic complexes and two mobile electron carriers that work in a …

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

Mutations in GBA1, the gene encoding the lysosomal enzyme β-glucocerebrosidase
(GCase), which cause Gaucher's disease, are the most frequent genetic risk factor for …

The structural and functional organization of the mitochondrial respiratory chain (MRC)
remains intensely debated. Here, we show the co-existence of two separate MRC …

Mitochondria are one of the most exhaustively investigated organelles in the cell and most
attention has been paid to the components of the mitochondrial electron transport chain …

ATP, the universal energy currency in all living cells, is mainly synthesized in mitochondria
by oxidative phosphorylation (OXPHOS). The final and rate limiting step of the respiratory …

In the final steps of energy conservation in aerobic organisms, free energy from electron
transfer through the respiratory chain is transduced into a proton electrochemical gradient …

Respiratory chains are crucial for cellular energy conversion and consist of multi‐subunit
complexes that can assemble into supercomplexes. These structures have been intensively …

Deep understanding of the pathophysiological role of the mitochondrial respiratory chain
(MRC) relies on a well-grounded model explaining how its biogenesis is regulated. The lack …

The mitochondrial translation machinery highly diverged from its bacterial counterpart. This
includes deviation from the universal genetic code, with AGA and AGG codons lacking …