Biochemical and ultrastructural findings in Epstein‐Barr virus‐transformed lymphoid cell lines from type 1 Gaucher disease

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.

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