Question

Acyl-CoA dehydrogenase deficiencies are diseases related to the impaired ability to oxidize fatty acids via beta oxidation. Symptoms of these acyl-CoA dehydrogenase deficiencies include hypoketosis [low blood levels of ketone bodies], hypoglycemia [low blood glucose], and hyperammonemia [increased blood levels of ammonia] during long periods of fasting.

Please explain why patients with acyl-coA dehydrogenase deficiencies have bouts of hypoketosis  [1- 2 sentences is sufficient]. (Please type answer, thanks!)

(BioChem)

Answer 1

Acyl-CoA dehydrogenase plays crucial role in the first step in the beta-oxidation of the fatty acids where it converts Acyl-CoA to α,β enoyl CoA which further goes into the reaction and converts into multiple Acetyl CoA in the last step. This Acetyl CoA produces ketone bodies in the liver mitochondria and maintain the amount of the ketone bodies in the blood. But when the former enzyme became nonfunctional then the  β-oxidation does not occur, for this reason no acetyl CoA are there to produce ketone bodies which leads to hypoketosis.