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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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