A 18-Month-Old Boy Is Brought to the Office Due to Developmental

A 18-month-old boy is brought to the office due to developmental delay and failure to thrive.  his mother states that he has 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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