Bchintermediate
Match each glycogen storage disease with its deficient enzyme: Glycogen Storage Diseases: 1. Pompe disease (Type II) 2. Cori disease (Type III) 3. McArdle disease (Type V) 4. Hers disease (Type VI) Enzymes: A. Lysosomal alpha-1,4-glucosidase (acid maltase) B. Glycogen debranching enzyme C. Skeletal muscle glycogen phosphorylase D. Liver glycogen phosphorylase
Answer
1-A, 2-B, 3-C, 4-D
Explanation
### The pairs
| Item | Matches | Why |
|---|---|---|
| Pompe disease | Lysosomal alpha-1,4-glucosidase | This enzyme degrades glycogen within lysosomes; its deficiency causes lysosomal glycogen accumulation. |
| Cori disease | Debranching enzyme | Failure to remove branch points produces limit-dextrin accumulation. |
| McArdle syndrome | Muscle glycogen phosphorylase | Muscle cannot mobilize glycogen normally for exercise. |
| Hers disease | Liver glycogen phosphorylase | The liver enzyme deficiency impairs hepatic glycogen breakdown. |
### Terms
- **Lysosome**: A cell compartment containing degradative enzymes.
- **Limit dextrin**: A branched glycogen remnant left when phosphorylase cannot proceed past branches.
### Remember
Identify the deficient enzyme and tissue to distinguish these disorders.
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