An individual suffers from a defect in one of the enzymes required for the synthesis of carnitine. If this individual does not have adequate intake of carnitine in the diet and is fasting, which of the following would be the most likely observation in this person compared to conditions of normal carnitine intake?

Question

Quizplus · Accepted Answer

Carnitine is required for the transport of fatty acyl-CoAs from the cytosol into the mitochondria for oxidation. Limiting levels of carnitine would, therefore, result in reduced rates of mitochondrial b-oxidation of fatty acids. A reduced capacity for mitochondrial oxidation would ultimately result in a reduced capacity for cells to take fatty acids from the blood ultimately leading to hyperlipidemia. Since fatty acid oxidation would be impaired there would be reduced ketogenesis occurring and less energy production would restrict gluconeogenesis.

An Individual Suffers from a Defect in One of the Enzymes