Question

In i-cell disease, an enzyme in the endomembrane system that normally adds phosphorylated sugar groups to proteins is lacking, and the proteins are not targeted to the lysosomes as they would be in normal cells. the "i" stands for inclusion bodies that appear in the cells. what do you think these inclusions are, and why do they accumulate?

Answer 1

Some substance need to be processed before dumped. If they are not processed, the substance could stuck inside the cell because there is no pump to put them outside. In i-cell disease, the enzyme that needed to degrade the substance is lacking. This make the cell unable to degrade the substance , make it stuck and accumulated inside the cell. These inclusion should be the substance that can't be degraded.