What happens when you are acyl CoA dehydrogenase deficient?

Without acyl CoA dehydrogenase to initiate the first step of mitochondrial beta-oxidation, your ability to metabolize fats is inhibited (1). That is, the enzyme—one of four depending on fatty acid chain lengths—catalyzes the formation of the double bond between alpha- and beta-carbons, which then are degraded to two-carbon acetyl CoA units (1p159).

Because the body relies on the production of acetyl CoA from its storage of fat for energy, the lack of acetyl CoA dehydrogenase and in its capacity to produce beta-oxidation causes the body to end up in a hypoglycemic (no glucose) and hypoketotic state (no ketones from oxidation of fatty acids) (1p159;2-3).

Reference List
1. Gropper SS, Smith JL, Groff JL. Advanced Nutrition and Human Metabolism. Belmont, CA: Thomson Wadsworth, 2009.
2. PerkinElmer Genetics. Multiple Acyl-CoA Dehydrogenase Deficiency. Available at: http://www.perkinelmergenetics.com/MultipleAcylCoADehydrogenaseDeficiency.htm.
3. STAR-G. Genetic Fact Sheet for Parents: Fatty Acid Oxidation Disorders. Available at: http://www.newbornscreening.info/Parents/fattyaciddisorders/VLCADD.html.

Published by David Despain, MS, CFS

David is a science and health writer living on Long Island, New York. He's written for a variety of publications including Scientific American, Outside Online, the American Society for Nutrition's (ASN) Nutrition Notes Daily, and Institute of Food Technologists' (IFT) Food Technology magazine and Live! blog. He's also covered new findings reported at scientific meetings including Experimental Biology, AAAS, AOCS, CASW, Sigma Xi, IFT, and others on his personal blog "Evolving Health." David is also an active member of organizations including the National Association of Science Writers (NASW), the American Association for the Advancement of Science (AAAS), the American Society for Nutrition, the Institute of Food Technologists, and the National Audubon Society. David has a master's degree in human nutrition from the University of Bridgeport, and a bachelor's degree in English from University of Illinois at Springfield. He also earned his Certified Food Scientist credential from the Institute of Food Technologists.

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