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I-Cell Disease (Also Identified as Mucolipidosis Type II) Is Characterized

Question 1

Multiple Choice

I-cell disease (also identified as mucolipidosis type II) is characterized by the presence of inclusion bodies in fibroblasts (hence the derivation of the term I-cell) , severe psychomotor retardation, corneal clouding, and dysostosis multiplex. These symptoms arise from a defect in the targeting of lysosomal enzymes due to an inability to carry out which of the following processes?


A) produce mannose-6-phosphate modifications in lysosomal enzymes
B) recycle the lysosomal receptor for mannose 6-phosphate present on lysosomal enzymes
C) remove mannose 6-phosphates from lysosomal enzymes prior to their transport to the lysosomes
D) synthesize the mannose 6-phosphate receptor found in lysosomes
E) transport mannose-6-phosphate receptors to lysosomes

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