Abstract
Severe and prolonged impairment of mitochondrial beta-oxidation leads to microvesicular steatosis, and, in severe forms, to liver failure, coma and death. Impairment of mitochondrial beta-oxidation may be either genetic or acquired, and different causes may add their effects to inhibit beta-oxidation severely and trigger the syndrome. Drugs and some endogenous compounds can sequester coenzyme A and/or inhibit mitochondrial beta-oxidation enzymes (aspirin, valproic acid, tetracyclines, several 2-arylpropionate anti-inflammatory drugs, amineptine and tianeptine); they may inhibit both mitochondrial beta-oxidation and oxidative phosphorylation (endogenous bile acids, amiodarone, perhexiline and diethylaminoethoxyhexestrol), or they may impair mitochondrial DNA transcription (interferon-alpha), or decrease mitochondrial DNA replication (dideoxynucleoside analogues), while other compounds (ethanol, female sex hormones) act through a combination of different mechanisms. Any investigational molecule should be screened for such effects.
MeSH terms
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Animals
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Anti-Bacterial Agents / administration & dosage
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Anti-Bacterial Agents / pharmacology
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Anti-Bacterial Agents / therapeutic use
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Anti-Inflammatory Agents / administration & dosage
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Anti-Inflammatory Agents / pharmacology
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Anti-Inflammatory Agents / therapeutic use
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Bile Acids and Salts / administration & dosage
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Bile Acids and Salts / pharmacology
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Bile Acids and Salts / therapeutic use
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Chemical and Drug Induced Liver Injury*
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DNA Replication / drug effects
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DNA Replication / genetics
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DNA, Mitochondrial / genetics
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DNA, Mitochondrial / metabolism
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Electron Transport
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Ethanol / toxicity
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Fat Necrosis / chemically induced
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Fatty Liver / etiology
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Female
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Hormones / administration & dosage
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Hormones / pharmacology
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Hormones / therapeutic use
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Humans
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Liver Diseases / metabolism
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Metabolism, Inborn Errors / etiology
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Mitochondria, Liver / drug effects*
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Mitochondria, Liver / metabolism
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Mitochondria, Liver / pathology
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Oxidation-Reduction
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Oxidative Phosphorylation
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Pregnancy
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Pregnancy Complications / etiology
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Reye Syndrome / etiology
Substances
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Anti-Bacterial Agents
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Anti-Inflammatory Agents
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Bile Acids and Salts
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DNA, Mitochondrial
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Hormones
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Ethanol