BIO NMDCAT Cytoplasmic Organelles
nmdcat.online July 4, 2026

52. A molecular mutation that renders the enzyme phosphotransferase nonfunctional prevents the formation of the M6P tag, leading directly to the clinical manifestation of

A. The accumulation of glycogen in the smooth ER channels
B. The mistargeting and extracellular secretion of lysosomal enzymes
C. The permanent arrest of transport from the rough ER
D. The structural collapse of the nuclear envelope matrix

📝 Explanation

Without the mannose-6-phosphate (M6P) tag, lysosomal enzymes miss their sorting receptors in the trans-Golgi, routing into the default secretory pathway instead (I-cell disease).

📖 Additional Information

  • The accumulation of glycogen in the smooth ER channels
  • The mistargeting and extracellular secretion of lysosomal enzymes
  • The permanent arrest of transport from the rough ER
  • The structural collapse of the nuclear envelope matrix

Without the mannose-6-phosphate (M6P) tag, lysosomal enzymes miss their sorting receptors in the trans-Golgi, routing into the default secretory pathway instead (I-cell disease).

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