Inherited defects that impair b-oxidation of fatty acids at different stages of the chain-shortening process have been identified. A long-chain acyl-CoA dehydrogenase, a medium-chain acyl-CoA dehydrogenase, and a short-chain acyl-CoA dehydrogenase are required to attach acyl-CoA chains of greater than 12 carbons, 6-12 carbons, and 4-6 carbons, respectively. Patients with one of these defects generally do well simply by avoiding prolonged periods of starvation.
Question:
Medium-chain acyl-CoA deficiency was first recognized in 1982 and is now thought to be a relatively common inheritable metabolic disease. If a patient with a defect in the medium chain acyl-CoA dehydrogenase is starved, the patient will develop hypoglycemia and have symptoms including vomiting, lethargy and frequently coma. Hypoglycemia would normally be accompanied by an elevation in ketone body production, but this does not occur in these patients. Why do these patient fail to generate ketone bodies as an alternative fuel? and what is the likely cause of hypoglycemia?
Inherited defects that impair b-oxidation of fatty acids at different stages of the chain-shortening process have been identified. A long-chain acyl-CoA dehydrogenase, a medium-chain acyl-CoA dehydrogenase, and a short-chain acyl-CoA dehydrogenase are required to attach acyl-CoA chains of greater than 12 carbons, 6-12 carbons, and 4-6 carbons, respectively. Patients with one of these defects generally do well simply by avoiding prolonged periods of starvation.
Question:
Medium-chain acyl-CoA deficiency was first recognized in 1982 and is now thought to be a relatively common inheritable metabolic disease. If a patient with a defect in the medium chain acyl-CoA dehydrogenase is starved, the patient will develop hypoglycemia and have symptoms including vomiting, lethargy and frequently coma. Hypoglycemia would normally be accompanied by an elevation in ketone body production, but this does not occur in these patients. Why do these patient fail to generate ketone bodies as an alternative fuel? and what is the likely cause of hypoglycemia?
Related textbook solutions
Basic Chemistry
Principles of Chemistry Molecular Approach
Chemistry: Structure and Properties
Principles of Chemistry Molecular Approach
Chemistry: A Molecular Approach
Chemistry: A Molecular Approach
Principles of Chemistry: A Molecular Approach
