Application | Comment | Organism |
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medicine | in medium-chain acyl-CoA dehydrogenase deficiency, the affected patients predominantly present high levels of octanoic and decanoic acids and their glycine and carnitine by-products in tissues and body fluids. Treatment of healthy brain mitochondrial preparations with octanoic and decanoic acid markedly increases state 4 respiration and diminishes state 3 respiration as well as the respiratory control ratio, the mitochondrial membrane potential and the matrix NAD(P)H levels. Decanoic acid-elicited increase in oxygen consumption in state 4 respiration is partially prevented by atractyloside. Octanoic and decanoic acid also reduce ADP/O ratio, CCCP-stimulated respiration and the activities of respiratory chain complexes.The major accumulating fatty acids in MCADD may act as uncouplers of oxidative phosphorylation and as metabolic inhibitors. Decanoic acid, but not octanoic acid, provokes a marked mitochondrial swelling and cytochrome c release from mitochondria, reflecting a permeabilization of the inner mitochondrial membrane | Rattus norvegicus |
Localization | Comment | Organism | GeneOntology No. | Textmining |
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mitochondrion | - |
Rattus norvegicus | 5739 | - |
Organism | UniProt | Comment | Textmining |
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Rattus norvegicus | - |
- |
- |
Source Tissue | Comment | Organism | Textmining |
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brain | - |
Rattus norvegicus | - |