A 2-Year-Old Boy Is Being Evaluated at the Office for Failure

A 2-year-old boy is being evaluated at the office for failure to thrive and developmental delay.  Medical history is significant for recurrent ear infections since 6 months of age.  Physical examination shows corneal clouding, hepatosplenomegaly, and restricted joint mobility.  Further evaluation shows deficient phosphorylation of mannose residues on certain glycoproteins in the Golgi apparatus.  In unaffected patients, these proteins are normally transported to which of the following cellular locations?

A) Endoplasmic reticulum
B) Extracellular space
C) Lysosome
D) Mitochondria
E) Plasma membrane

Correct Answer:

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