Question

In l-cell disease, an enzyme in the endomembrane system that normally adds phosphorylated sugar groups to proteins is lacking. In normal cells, which are not missing this enzyme, such phosphorylated protiens are targeted to the lysosome. In l-cell disease inclusion bodies appear in the cells. What do you think these inclusion bodies are made of and why do they accumulate?

Answer 1

Answer:-

In I-cell disease inclusion bodies appear in the cells. Firstly I-cell disease is also called inclusion cell disease. It is an autosomal recessive disorder. In this disease, an enzyme known as phosphotransferase does not perform it's function properly which is basically involved in the transfer of phosphorylated sugar group to specific proteins on which mannose residues are present and that results in Mannose-6-phosphate which act as a marker for the proteins in order to targeted to the lysosome. But as there is the presence of defective enzyme in I-cell disease, the whole function would get interupted as the lysosomes are not able to perform it's function due to which normal breakdown of the substances would not occur as proteins are not present. These substances generally include lipids, proteins, oligosaccharides, cellular debris etc. These substances then accumulate within the lysosome and results in inclusion bodies as they cannot be deteriorate chemically.

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