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What is 'I' cells disease is due to defect in:- and explain in detail?
I cell disease (Inclusion cell disease) Pa of the lysosomal storage disease family Defective phosphotransferase (uridine-diphosphate-N-acetylglucosamine 1-phosphotransferase) an enzyme of the Golgi apparatus Enzyme that phosphorylates mannose residues of glycoproteins to allow their delivery to lysosomes from Golgi Apparatus =Features:- Protein targeting disorder Resembles Hurler syndrome Neurological deterioration mucopolysacchariduria is not present,It is not a mucopolysaccharidosis, it is mucolipidosis - glycolipids are accumulated Marked intracellular deficiency of a number of lysosomal hydrolases and elevation of these enzymes in plasma.