Metabolism USMLE Step 1 Practice Question
A previously normal child begins deteriorating developmentally at about 6 months of age. She is seen by an ophthalmologist because she no longer responds to visual stimuli. Ophthalmoscopic examination finds a region of cells around the fovea centralis with a cloudy, milky appearance. The enzyme that is deficient in this child normally carries out which of the following functions?
Answer choices
- ADegradation of glycogen in muscle
- BSynthesis of glycoproteins in the liver
- CDegradation of mucopolysaccharides in bone marrow
- DDegradation of glycolipids in the brainCorrect answer
- ESynthesis of peptides in the spleen
- FDegradation of sphingomyelin in the liver
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