Question

A number of genetic deficiencies in acyl CoA dehydrogenases have been described. This deficiency presents early in life after a period of fasting. Symptoms include vomiting, lethargy, and sometimes coma. Not only is the blood concentration of glucose low (hypoglycemia), but starvation-induced ketosis is absent. Provide a biochemical explanation for these last two observations.

Answer 1

   Acyl coA dehydrogenase is an enzyme used in the breaking down of the stored fat in the body. Fat is used as a source of energy during fasting state. So during fasting state, the fat is broken down to from glucose and used as energy. In this disease of deficiency of acyl-coA dehydrogenase, there is absence of catabolism of stored fat and that will result in hypoglycemia. Also starvation induced ketosis is absent because there is no fat break down for forming the ketone bodies. So the symptoms caused by this enzyme deficiency is directly due to hypoglycaemia. For our normal body functioning brain needs 120 g of glucose daily and this glucose is used in the normal functioning of the neurons in the brain. When there is a severe decrease in total body glucose, it causes cerebral hypoglycaemia and the neurons fail to perform it's function in controlling the body. According to the level of glucose present, the person will manifest lethargy primarily and when the hypoglycaemic state prolongs, coma is also manifested if the glucose level fall below 50 mg/ dl approximately.