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.

a.) Please explain why patients with acyl-coA dehydrogenase deficiencies have bouts of hypogycemia  [1- 2 sentences is sufficient].

b.) Please explain why patients with acyl-coA dehydrogenase deficiencies have bouts of hyperammonemia [1- 2 sentences is sufficient].

(BioChem) (Please type answer, thanks!)

Answer 1

a) Why patients with acyl-CoA dehydrogenase deficiency have hypoglycaemia?

Lack of acyl-CoA dehydrogenase leads to inability to oxidize fatty acids thereby cells have to depend on other sources of fuels like carbohydrates and amino acids to produce energy to survive. This dependence of cells on carbohydrates for energy reduces blood glucose level (hypogylcemia) because most of the blood glucose is being utilized by cells to generate energy.

b) Why patients with acyl-CoA dehydrogenase deficiency have hyperammonemia?

Lack of acyl-CoA dehydrogenase leads to inability to oxidize fatty acids thereby cells have to depend on other sources of fuels like carbohydrates and amino acids to produce energy to survive. This dependence of cells on amino acids for energy increases blood ammonia level (hyperammonemia) because amino acids are constantly being degraded to generate energy. Constant degradation of amino acids generates more ammonia and urea.