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- W2071132014 abstract "Abstract Fusions have been carried out between fibroblasts from patients with “I-cell” disease and enucleated human fibroblasts with a single lysosomal enzyme deficiency derived from patients with GM1-gangliosidosis, Sandhoff disease and mannosidosis. Pure cytoplasts were obtained using cytochalasin B treatment followed by fluorescence activated cell sorting. After fusion with whole “I-cells”, the cybrid populations showed a restoration of deficient lysosomal enzyme activity and also the abnormal electrophoretic pattern characteristic for the residual hexosaminidase activity in “I-cells” was found to be corrected. The results described in this paper indicate that the defective post-translational modification, which is responsible for the multiple lysosomal enzyme deficiency, can be corrected by a factor that is stable for at least three days in enucleated cells. During this period the cytoplasmic factor can act without the need of de novo synthesis but the absence of correction in in vitro experiments shows that cellular integrity is required." @default.
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- W2071132014 date "1980-06-01" @default.
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- W2071132014 title "Fusion with enucleated fibroblasts corrects “I-cell” defect" @default.
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- W2071132014 doi "https://doi.org/10.1016/0014-4827(80)90460-7" @default.
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