Article
Food withdrawal lowers energy expenditure and induces inactivity in long-chain fatty acid oxidation-deficient mouse models.
FASEB journal : official publication of the Federation of American Societies for Experimental Biology - 1 Jul 2014
Diekman Eugene F, van Weeghel Michel, Wanders Ronald J A, Visser Gepke, Houten Sander M
Abstract excerpt
Very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency is an inherited disorder of mitochondrial long-chain fatty acid β-oxidation (FAO). Patients with VLCAD deficiency may present with hypoglycemia, hepatomegaly, cardiomyopathy, and myopathy. Although several mouse models have been developed to aid in the study of the pathogenesis of long-chain FAO defects, the muscular phenotype is underexposed. To address...
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