KROK 1 2010 – Question 117 · KROK (Medicine) · Biological Chemistry
Laboratory examination of a child revealed increased concentration of leucine, valine, isoleucine and their ketoderivatives in blood and urine. Urine smelt of maple syrup. This disease is characterized by the deficit of the following enzyme:
- A. Glucose-6-phosphatase
- B. Phosphofructokinase
- C. Dehydrogenase of branched amino acids
- D. Aminotransferase
- E. Phosphofructomutase
Hint
Both the parent compounds and their keto derivatives accumulate, so the block must lie beyond the step that makes the keto derivatives; ask which reaction comes next in the degradation pathway.
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