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Biochemical and ultrastructural findings in Epstein-Barr virus-transformed lymphoid cell lines from type 1 Gaucher
Biology of the Cell
|January 1, 1987
Abstract:
Lymphoid cell lines established by Epstein-Barr Virus (EBV)-transformation of peripheral blood B-lymphocytes from patients affected with type 1 Gaucher disease showed a severe deficiency of glucosylceramide-beta-glucosidase activity (residual activity around 15%-30% of control activity). Ultrastructural investigations showed, in these lymphoid cell lines from type 1 Gaucher disease, the presence of numerous membrane-bound inclusion bodies characteristic of Gaucher cells.