what is responsible for the deficiency in I cell disease patients in which the l

Question

what is responsible for the deficiency in I cell disease patients in which the lysosomal enzymes do not carry the normal mannose phosphate residues that target them to lysosomes? what is responsible for the deficiency in I cell disease patients in which the lysosomal enzymes do not carry the normal mannose phosphate residues that target them to lysosomes? what is responsible for the deficiency in I cell disease patients in which the lysosomal enzymes do not carry the normal mannose phosphate residues that target them to lysosomes?

Explanation / Answer

It is caused due to deficiency of number of lysosomal hydrolase Uridine diphosphate-N-acetylglucosamine1-phosphotransferase. this enzyme phosphorylates mannose residue of glycoprotein to allow their delivery to lysosome.

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