Bchbasic
Von Gierke disease (glycogen storage disease type Ia) is caused by an inherited deficiency of the enzyme _____.
Answer
glucose-6-phosphatase
Explanation
### Why this is right
Von Gierke disease, or type I glycogenosis, results from absent or very low glucose-6-phosphatase activity, especially in liver and kidney tissues. Glycogen can accumulate but cannot be used normally to release free glucose.
### The concept
The defect blocks the final conversion of glucose-6-phosphate to glucose. This impairs blood-glucose maintenance during fasting or hormonal stimulation of glycogen breakdown.
### Terms
- **Glycogenosis**: A disorder involving glycogen metabolism.
- **Glucose-6-phosphatase**: An enzyme that releases free glucose from glucose-6-phosphate.
### Remember
Type I glycogenosis is the glucose-6-phosphatase deficiency.
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