Pompe's disease is characterized by which enzymatic deficiency?

Pompe's disease is a type of glycogen storage disease caused by a deficiency in the enzyme alpha-1,4-glucosidase, which is also known as acid maltase. This enzyme is responsible for breaking down glycogen into glucose in the lysosomes. When there is a deficiency of this enzyme, glycogen accumulates within the lysosomes leading to various pathological effects, particularly in muscle tissues.

The accumulation of glycogen in Pompe's disease primarily affects skeletal muscle and heart muscle, leading to progressive weakness and heart dysfunction. The infantile form of Pompe's disease presents early, often with severe cardiac involvement and muscle weakness, while the late-onset form may have a slower progression and be less severe, but still affects muscle function.

In contrast, other options refer to different enzymatic deficiencies associated with other glycogen storage diseases. For example, a deficiency in the debranching enzyme relates to Cori disease, while glycogen phosphorylase deficiency is linked to McArdle disease. A deficiency in branching enzyme causes Anderson disease. Each of these conditions has its unique clinical manifestations and implications, but they do not involve alpha-1,4-glucosidase, which is specifically tied to Pompe's disease.