**Core Concept**
Hereditary fructose intolerance (HFI) is a rare genetic disorder characterized by the inability to metabolize fructose due to a deficiency of the enzyme aldolase B. This enzyme is crucial for the breakdown of fructose-1-phosphate to glyceraldehyde and dihydroxyacetone phosphate in the liver.
**Why the Correct Answer is Right**
Aldolase B catalyzes the conversion of fructose-1-phosphate to glyceraldehyde and dihydroxyacetone phosphate, which are then further metabolized to glucose and pyruvate. In individuals with HFI, the lack of aldolase B leads to the accumulation of fructose-1-phosphate in the liver, causing hypoglycemia, vomiting, and other systemic symptoms. The deficiency of aldolase B is caused by mutations in the ALDOB gene, leading to the production of a non-functional enzyme.
**Why Each Wrong Option is Incorrect**
**Option A:** Glucose-6-phosphatase is the enzyme deficient in von Gierke's disease, not hereditary fructose intolerance.
**Option B:** Fructokinase is the enzyme involved in the initial phosphorylation of fructose to fructose-1-phosphate, but its deficiency leads to a different condition, essential fructosuria.
**Option C:** Aldolase A is an isoenzyme of aldolase B and is primarily involved in glycolysis in muscle tissue, not in the metabolism of fructose.
**Clinical Pearl / High-Yield Fact**
The symptoms of hereditary fructose intolerance can be alleviated by a fructose-free diet, and patients should be counseled to avoid consuming foods containing fructose, such as fruits, fruit juices, and honey.
**Correct Answer:** C. Aldolase B
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