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Targeted disruption of mouse long-chain acyl-CoA dehydrogenase gene reveals crucial roles for fatty acid oxidation.

http://www.ncbi.nlm.nih.gov/pubmed/9861014

Abnormalities of fatty acid metabolism are recognized to play a significant role in human disease, but the mechanisms remain poorly understood. Long-chain acyl-CoA dehydrogenase (LCAD) catalyzes the initial step in mitochondrial fatty acid oxidation (FAO). We produced a mouse model of LCAD deficiency with severely impaired FAO. Matings between LCAD +/- mice yielded an abnormally low number of LCAD +/- and -/- offspring, indicating frequent gestational loss. LCAD -/- mice that reached birth appeared normal, but had severely reduced fasting tolerance with hepatic and cardiac lipidosis, hypoglycemia, elevated serum free fatty acids, and nonketotic dicarboxylic aciduria. Approximately 10% of adult LCAD -/- males developed cardiomyopathy, and sudden death was observed in 4 of 75 LCAD -/- mice. These results demonstrate the crucial roles of mitochondrial FAO and LCAD in vivo.

Pubmed ID: 9861014 RIS Download

Mesh terms: Acyl-CoA Dehydrogenase, Long-Chain | Animals | Disease Models, Animal | Fatty Acids, Nonesterified | Humans | Lipid Metabolism, Inborn Errors | Liver | Mice | Mice, Knockout | Mitochondria, Liver | Muscle, Skeletal | Substrate Specificity

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Associated grants

  • Agency: NCI NIH HHS, Id: P30CA-13148
  • Agency: NIDDK NIH HHS, Id: R01-DK45482
  • Agency: NCRR NIH HHS, Id: R01-RR02599

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