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

Complex I deficiency is the most frequent mitochondrial disorder presenting in childhood,
accounting for up to 30% of cases. As with many mitochondrial disorders, complex I …

Leigh syndrome (LS) is a severe manifestation of mitochondrial disease in children and is
currently incurable. The lack of effective models hampers our understanding of the …

Apoptosis‐inducing factor (AIF) is a mitochondrial flavoprotein that, after apoptosis induction,
translocates to the nucleus where it participates in apoptotic chromatinolysis. Here, we show …

To study effects of mitochondrial complex I (CI, NADH: ubiquinone oxidoreductase)
deficiency, we inactivated the Ndufs4 gene, which encodes an 18 kDa subunit of the 45 …

Mitochondria are essential for cellular bioenergetics by way of energy production in the form
of ATP through the process of oxidative phosphorylation. This crucial task is executed by five …

Mitochondria are small cellular constituents that generate cellular energy (ATP) by oxidative
phosphorylation (OXPHOS). Dysfunction of these organelles is linked to a heterogeneous …

Inhibition of mitochondrial complex I is one of the leading hypotheses for dopaminergic
neuron death associated with Parkinson's disease (PD). To test this hypothesis genetically …

Respiratory complex I (NADH: ubiquinone oxidoreductase) is essential for cellular energy
production and NAD+ homeostasis. Complex I mutations cause neuromuscular …

Emerging evidence suggest that macrophage and osteoclast are two competing
differentiation outcomes from myeloid progenitors. In this review, we summarize recent …