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Pompe's Disease

APBIO-GOSEXZ

Pompe's disease is a hereditary disorder caused by a DNA mutation in which the individual is unable to correctly synthesize functional lysosomal enzyme alpha-glucosidase (GAA), the enzyme that normally allows the cells to break down glycogen for energy.

How could a DNA mutation of this type produce the characteristics seen in those affected with this disorder?

A

The altered lysosomal enzymes catalyze the excessive breakdown of glycogen, causing the cells to run out of energy and die.

B

The mutated form of the DNA would produce an enzyme that would not be transported to the lysosomes. It would instead break down glycogen in the nucleus.

C

The mutated form of the enzyme cannot effectively break down glycogen. Therefore, glycogen is stored in the body instead of being broken down for energy.

D

The altered lysosomal enzymes break down proteins, instead of glycogen. The glycogen is stored in the body, destroying muscles and organs.