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nmdcat.online July 3, 2026

88. The disease known as I-cell disease (mucolipidosis II) is a severe lysosomal storage disorder caused by a deficiency in the enzyme UDP-GlcNAc phosphotransferase. As a result of this molecular defect, lysosomal enzymes

A. Are synthesized with abnormal lipid tails
B. Lack the mannose-6-phosphate sorting tag and are constitutively secreted out of the cell
✓
C. Are permanently trapped inside the nuclear matrix
D. Undergo spontaneous conversion into structural proteoglycans

📝 Explanation

Without the phosphotransferase enzyme, lysosomal proteins lack the mannose-6-phosphate tag needed for sorting, causing them to be misdirected and secreted.

📖 Additional Information

  • Are synthesized with abnormal lipid tails
  • Lack the mannose-6-phosphate sorting tag and are constitutively secreted out of the cell
  • Are permanently trapped inside the nuclear matrix
  • Undergo spontaneous conversion into structural proteoglycans

Without the phosphotransferase enzyme, lysosomal proteins lack the mannose-6-phosphate tag needed for sorting, causing them to be misdirected and secreted.

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