Verified Solution Biology Cell: The Unit of Life

I-cell disease (Mucolipidosis II) is a rare inherited disorder caused by the inability of the Golgi apparatus to properly "tag" certain lysosomal enzymes with mannose-6-phosphate. This leads to these enzymes being secreted out of the cell instead of being delivered to lysosomes. What is the direct consequence within the cell?

6 views 1 helpful Updated Jul 21, 2026
Solution ✔ Verified
  • AAccumulation of undigested substrates in lysosomes, leading to cellular dysfunction.
  • BEnhanced synthesis of proteins due to increased enzyme availability in the cytoplasm.
  • CRapid breakdown of cellular waste products by the secreted enzymes.
  • DImpaired ATP production due to mitochondrial enzyme deficiency.
Explanation

If lysosomal enzymes are not correctly tagged and delivered to lysosomes, they cannot perform their function of breaking down cellular waste. This leads to the accumulation of undigested substrates within the lysosomes, causing cellular damage and dysfunction.

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