Multiple transfer of lysosomal enzymes from normal lymphocytes to I-cell disease fibroblasts.
Cells from patients with inherited lysosomal deficiency diseases can acquire the missing lysosomal enzyme by direct cell-to-cell transfer from normal lymphocytes. Cells from I-Cell Disease (Mucolipidosis type II; ICD) patients are simultaneously deficient in many lysosomal enzymes due to an inborn error of glycoprotein processing. In this study we show that such cells acquire high levels of several of the missing lysosomal enzymes when they are cultured in contact with lymphocytes. Moreover, the present results also show that enzyme levels in the donor lymphocytes are not depleted but increase during cell contact with the fibroblasts.
D000818:Animals; D002450:Cell Communication; D002478:Cells, Cultured; D005347:Fibroblasts / Q000201:enzymology; D005966:Glucuronidase / Q000032:analysis; D008214:Lymphocytes / Q000201:enzymology*; D008247:Lysosomes / Q000201:enzymology*; D051379:Mice; D008808:Mice, Inbred CBA; D009081:Mucolipidoses / Q000201:enzymology*
D005966:Glucuronidase