Amino acid metabolism is implicated in which disease?: (A) Maple syrup urine disease, (B) Reye's syndrome, (C) Von Gierke's disease, (D) McArdle's diseases
Answer is A. OPTION 1: Maple syrup urine disease - It is inborn error of metabolism of branched chain amino acids Von Gierke's disease and McArdle's diseases are glycogen storage diseases. Reye's syndrome is an acute self - limiting metabolic insult resulting in generalized mitochondrial dysfunction due to inhibition of fatty acid oxidation.