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  • Title: I-cell disease: intracellular desialylation of lysosomal enzymes using an influenza virus vector.
    Author: Spritz RA, Coates PM, Lief FS.
    Journal: Biochim Biophys Acta; 1979 Jan 04; 582(1):164-71. PubMed ID: 760815.
    Abstract:
    It has been proposed that I-cell disease results from a primary deficiency of acid neuraminidase activity. Infection by influenza virus of fibroblasts from a patient with I-cell disease resulted in the production of abundant intracellular alpha2-3 neuraminidase activity. Despite electrophoretic evidence of desialylation of intracellular and fibroblast-secreted arylsulfatase (EC 3.1.6.1) and beta-hexosaminidase (EC 3.2.1.30) from the infected cells, there was no consequent alteration of the abnormal distribution of beta-hexosaminidase activity between the intracellular spaces characteristic of I-cell disease. This suggests that deficiency of alpha2,3 neuraminidase activity is not the primary biochemical defect in I-cell disease.
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