A 5- year-old boy suffered gastroenteritis for two days and was brought to emergency in the semiconscious state. Blood glucose at the time of admission was 45 mg/dl and his urine was negative for glucose and ketone bodies. Glucose was administered intravenously and his condition improved within 10 minutes. Subsequent laboratory investigations revealed elevated acylcarnitine residues. The child was diagnosed with an enzyme deficiency. The parents were instructed to feed the child more frequently.
Which of the following enzymes might be deficient?
A. Acyl Co A synthetase
B. Carnitine Acyl transferase-1
D. Carnitine Acyl transferase-2
E. Acyl co A dehydrogenase
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Elevated Acylcarnitine compounds and associated hypoglycemia- What is the defect?