Bchadvanced
A person has exercise intolerance, high muscle glycogen, and little or no blood lactate after exercise. Which deficiency best fits this pattern?
Answer
Muscle glycogen phosphorylase deficiency
Explanation
### Why this is right
This is the characteristic pattern of McArdle syndrome, type V glycogenosis. Muscle glycogen phosphorylase deficiency prevents normal mobilization of muscle glycogen during exercise, limiting substrate supply for glycolysis and lactate production.
### The concept
The stored glycogen is abundant but metabolically difficult to access. This explains why a storage disorder can produce high tissue glycogen alongside impaired exercise performance.
### The other options
- **Liver glucose-6-phosphatase deficiency**: This causes von Gierke disease and disrupts blood-glucose release.
- **Lysosomal alpha-1,4-glucosidase deficiency**: This causes Pompe disease and lysosomal glycogen accumulation.
- **Liver glycogen phosphorylase deficiency**: This is Hers disease, involving hepatic glycogen breakdown.
### Terms
- **McArdle syndrome**: Muscle glycogen phosphorylase deficiency.
- **Glycolysis**: The pathway that breaks down glucose to pyruvate or lactate.
### Remember
Exercise symptoms plus high muscle glycogen point to impaired muscle glycogen mobilization.
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