Mitochondrial β-oxidation of fatty acids generates acetyl-coA, NADH and FADH 2. Acyl-coA
synthetases catalyze the binding of fatty acids to coenzyme A to form fatty acyl-coA …

Electron transfer flavoprotein (ETF) is an enzyme with orthologs from bacteria to humans.
Human ETF is nuclear encoded by two separate genes, ETFA and ETFB, respectively. After …

Single‐point mutation in genome, for example, single‐nucleotide polymorphism (SNP) or
rare genetic mutation, is the change of a single nucleotide for another in the genome …

Multiple acyl-CoA dehydrogenase deficiencies (MADDs) are a heterogeneous group of
metabolic disorders with combined respiratory-chain deficiency and a neuromuscular …

In mammalian mitochondria, protein methylation is a relatively uncommon post-
transcriptional modification, and the extent of the mitochondrial protein methylome, the …

Reactive oxygen species production by mitochondrial enzymes plays a fundamental role
both in cellular signaling and in the progression of dysfunctional states. However, sources of …

During the last two decades the realization has emerged that the phenotype of the majority
of inherited genetic diseases, including inborn errors of metabolism, cannot be predicted by …

Hypoxia poses a stress to cells and decreases mitochondrial respiration, in part by electron
transport chain (ETC) complex reorganization. While metabolism under acute hypoxia is …

We recently demonstrated that in addition to its protective effect on pancreatic and adrenal
biosynthesis, antioxidant properties of sericin decrease blood cholesterol levels and …

A newly recognized third fundamental mechanism of energy conservation in biology,
electron bifurcation, uses free energy from exergonic redox reactions to drive endergonic …