Question:
In Hurler syndrome, which glycosaminoglycans accumulate and cause organ enlargement?
In Hurler syndrome, which glycosaminoglycans accumulate and cause organ enlargement?
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Hurler syndrome = alpha-L-iduronidase deficiency = accumulation of heparan sulfate and dermatan sulfate.
Updated On: May 14, 2026
- Keratan sulfate and dermatan sulfate
- Keratan sulfate and heparan sulfate
- Heparan sulfate and dermatan sulfate
- Keratan sulfate and hyaluronic acid
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The Correct Option is C
Solution and Explanation
Concept:
Hurler syndrome is a mucopolysaccharidosis caused by deficiency of alpha-L-iduronidase. It leads to accumulation of specific glycosaminoglycans.
Step 1: Recall enzyme deficiency.
Hurler syndrome is due to deficiency of: \[ \alpha\text{-L-iduronidase} \]
Step 2: Identify accumulated substances.
The accumulated glycosaminoglycans are: \[ \text{Heparan sulfate and dermatan sulfate} \]
Step 3: Relate with organ enlargement.
Their accumulation causes coarse facies, organomegaly and skeletal abnormalities.
Step 1: Recall enzyme deficiency.
Hurler syndrome is due to deficiency of: \[ \alpha\text{-L-iduronidase} \]
Step 2: Identify accumulated substances.
The accumulated glycosaminoglycans are: \[ \text{Heparan sulfate and dermatan sulfate} \]
Step 3: Relate with organ enlargement.
Their accumulation causes coarse facies, organomegaly and skeletal abnormalities.
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