Describe how CI-M6PR deliveries newly-synthesized enzymes to the lysosome? You may use diagrams to explain or...

Describe how CI-M6PR deliveries newly-synthesized enzymes to the lysosome? You may use diagrams to explain or enhance your answer. What’s part of the responsible for the mechanism underlying I-cell disease.

Expert Solution

Previous knowledge to answer the question:

CI-M6PR stands for Cation-independent mannose-6-phosphate receptor.

I-cell disease stands for inclusion-cell disease, caused by a mutant GlcNAc phosphotransferase, an enzyme that transfers a GlcNAc-phosphate to proteins with an N-linked oligosaccharide that has a mannose residue.

Describe how CI-M6PR deliveries newly-synthesized enzymes to the lysosome?

The cation-independent mannose-6-phosphate receptor is vital for the transport of newly synthesized lysosomal enzymes to lysosomes, therefore degradation of defective proteins doesn't take place if there is a mutation in this receptor, and a lot of malfunction debris starts to accumulate. The CI-M6PR receptor binds to the new hydrolases in the Golgi apparatus and which serves as a signal to deliver them to pre-lysosomal compartments.

The part in this process responsible for the mechanism underlying I-cell disease is that in this disease there is a mutation in the GlcNAc phosphotransferase, enzyme in charge of phosphorylating some lysosomal hydrolases with mannose residues, therefore the signal of mannose-6-phosphate will be absent in patient with this diseases, and the lysosomal hydrolases won't be detected by the CI-M6PR receptor, and finally, therefore they won't be sent to the lysosomes, where they help in the degradation of defective proteins.

gladiator answered 1 year ago

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