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Rieske protein TTC19 UQCRFS1 complex III deficiency mitochondrial complex III mitochondrial disease mitochondrial quality control mitochondrial respiratory chain mouse model Animals Behavior Animal Disease Models Animal Electron Transport Complex III Female Genotype HeLa Cells Humans Iron-Sulfur Proteins Kinetics Male Membrane Proteins Mice Inbred C57BL Mice Knockout Mitochondria Mitochondrial Diseases Mitochondrial Proteins Motor Activity Nerve Degeneration Nervous System Phenotype Protein Binding Protein Stability Proteolysis Reactive Oxygen Species

Mitochondria are ubiquitous intracellular organelles found in almost all eukaryotes and involved in various aspects of cellular life, with a primary role in energy production. The interest in this organelle has grown stronger...

ATP production biogenesis of the respiratory chain mitochondrial disease mi-tochondrial electrochemical gradient mitochondrial potential mitochondrial proton pumping mitochondrial respiratory chain Oxidative Phosphorylation respiratory complex respiratory supercomplex

de novo lipogenesis Hepatic mitochondria Hypoxia mitochondrial respiratory chain Mitochondrial supercomplexes Homeostasis Humans Hypoxia Lipid Metabolism Lipogenesis Liver

Regulation of the mitochondrial respiratory chain biogenesis is a matter of great interest because of its implications for mitochondrial disease. One of the mitochondrial disease genes recently discovered associated to...

mitochondrial respiratory chain OXPHOS Rieske Fe-S protein TTC19 UQCRFS1 Mitochondria mitochondrial complex III mitochondrial disease Animals Cattle Chickens Electron Transport Complex III Membrane Proteins Mice Mice Knockout Mitochondria Mitochondrial Proteins Protein Binding Protein Structure Quaternary Protein Subunits

RESEARCH ARTICLE Hypoxia Hepatic mitochondria mitochondrial respiratory chain Mitochondrial supercomplexes de novo lipogenesis