krsma
Forum Elite
Topics: 40
Posts: 227
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What happens in I-cell disease?
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| Bela
Forum Guru
Topics: 76
Posts: 412
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It's the disease of empty lysosomes.
When the digestive enzymes for lysosomes are being processed in Golgi, lack of phosphotransferase won't make phosphorilation of mannose on these enzymes possible. So, these digestive enzymes, instead of going to lysosomes are excreted from the cell and they will travel wild around our body 
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La vita e bella!
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| gr_calif
Forum Junior
Topics: 3
Posts: 51
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also ,the Multiple enzyme deficiencies associated with I-cell disease lead to the accumulation of certain fatty substances (mucolipids) and certain complex carbohydrates (mucopolysaccharides) within the cells of many tissues of the body- :arrow: the characteristic finding is abnormal vacuolization or inclusions that appear in the cytoplasm of cells of mesenchymal origin, especially fibroblasts.
The symptoms of I-cell disease are similar to but more severe than those of Hurler syndrome :arrow: coarse facial features, skeletal abnormalities and mental retardation.
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| usmleasr
Forum Guru
Topics: 105
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8) good explanation thanks
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| gr_calif
Forum Junior
Topics: 3
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NO ACTUALLY THANKS TO U ALL ,
in the process i get to learn too
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