Question
A 7 month old baby is present with vomiting, lethargy, severe jaundice when weaning starts with fruit juice. Which enzyme is defective
| A. |
Sucrase |
| B. |
Fructokinase |
| C. |
Aldolase A |
| D. |
Aldolase B |
Show Answer
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Correct Answer � D Explanation |
 |
Answer- D
- The appearance of above clinical features on the initiation of fruit juice suggests a disorder of fructose metabolism.
- Hereditary fructose intolerance (HFI) is due to deficiency of the enzyme Aldolase B. Fructose-1-phosphate is cleaved to D-glyceraldehyde and dihydroxyacetone phosphate by aldolase B, an enzyme found in the liver