A patient with hereditary fructose intolerance is deficient in which of the following enzyme-
**Core Concept**
Hereditary fructose intolerance is a rare genetic disorder caused by a deficiency of the enzyme aldolase B, which is essential for the breakdown of fructose-1-phosphate in the liver. This enzyme deficiency leads to the accumulation of toxic metabolites, causing various systemic symptoms.
**Why the Correct Answer is Right**
Aldolase B is a fructose-specific enzyme that catalyzes the conversion of fructose-1-phosphate to glyceraldehyde and dihydroxyacetone phosphate in the liver. In individuals with hereditary fructose intolerance, the deficiency of aldolase B leads to the accumulation of fructose-1-phosphate, causing hypoglycemia, vomiting, and other systemic symptoms. The enzyme is specifically localized in the liver and kidney, where it plays a crucial role in fructose metabolism.
**Why Each Wrong Option is Incorrect**
**Option A:** This option is incorrect because fructokinase is another enzyme involved in fructose metabolism, but its deficiency leads to a different condition called essential fructosuria, which is a benign condition.
**Option B:** This option is incorrect because phosphofructokinase is an enzyme involved in glycolysis, not fructose metabolism. Its deficiency is associated with glycogen storage disease type VII.
**Option C:** This option is incorrect because glucose-6-phosphatase is an enzyme involved in glucose metabolism, and its deficiency leads to glycogen storage disease type I.
**Clinical Pearl / High-Yield Fact**
Hereditary fructose intolerance is a classic example of a metabolic disorder caused by a deficiency of a specific enzyme, highlighting the importance of understanding the biochemical pathways involved in human metabolism.
**Correct Answer: C. Aldolase B is the correct enzyme deficiency in hereditary fructose intolerance.**