How does intravenous enzyme-replacement therapy for Gaucher disease reduce the organ storage of glucosylceramide?
A)The enzyme increases the destruction of glucosamine-filled macrophages.
B)The drug acts as a cofactor, increasing the activity of the mutated enzyme.
C)The drug increases production of all blood cells in the bone marrow, including white blood cells, which have not stored glucosylceramide.
D)The enzyme is absorbed through the plasma membranes of affected cells and converts the stored glucosylceramide into its constituent molecules.

Question

Quizplus · Accepted Answer

In Gaucher disease there is a deficiency of the GBA gene product, an enzyme that normally breaks down glucosylceramide into sugar and fat that can be recycled for other metabolic uses. The enzyme deficiency results in the accumulation of glucosylceramide in macrophages and some other white blood cells. Enzyme replacement acts like the naturally occurring enzyme, breaking down glucosylceramide into the usual sugar and fats. Drugs for enzyme-replacement therapy are imiglucerase (Cerezyme), taliglucerase (ELELYSO), and velaglucerase (VPRIV),

How Does Intravenous Enzyme-Replacement Therapy for Gaucher Disease Reduce the Organ