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Medium-chain acyl-CoA dehydrogenase (MCAD) is an enzyme involved in β-oxidation and is encoded by the autosomal ACADM gene.  MCAD deficiency is caused by a mutation in ACADM and results in impaired ability to digest medium-chain fatty acids for energy and in their subsequent accumulation.  Which of the following interventions would best relieve the negative effects of MCAD deficiency?A.Mitochondrial gene therapyB.Supplementation with long-chain fatty acidsC.Prolonged periods of fastingD.A high-carbohydrate, low-fat diet

Question

Medium-chain acyl-CoA dehydrogenase (MCAD) is an enzyme involved in β-oxidation and is encoded by the autosomal ACADM gene.  MCAD deficiency is caused by a mutation in ACADM and results in impaired ability to digest medium-chain fatty acids for energy and in their subsequent accumulation.  Which of the following interventions would best relieve the negative effects of MCAD deficiency?A.Mitochondrial gene therapyB.Supplementation with long-chain fatty acidsC.Prolonged periods of fastingD.A high-carbohydrate, low-fat diet

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Solution

The best intervention to relieve the negative effects of MCAD deficiency would be D. A high-carbohydrate, low-fat diet.

Here's why:

A. Mitochondrial gene therapy - While this could theoretically correct the genetic defect causing MCAD deficiency, it's currently not a widely available or proven treatment.

B. Supplementation with long-chain fatty acids - This wouldn't help because the problem in MCAD deficiency is the inability to break down medium-chain fatty acids, not long-chain ones.

C. Prolonged periods of fasting - This would actually be harmful. Fasting increases the body's reliance on fatty acids for energy, which would exacerbate the problem in MCAD deficiency.

D. A high-carbohydrate, low-fat diet - This would be the best option. By providing more carbohydrates, the body can rely less on fatty acids for energy. And by limiting fat intake, you reduce the amount of medium-chain fatty acids that the body has to deal with.

This problem has been solved

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