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

作者信息

Maret A, Salvayre R, Livni N, Icart J, Vuillaume M, Douste-Blazy L

出版信息

Biol Cell. 1987;59(1):101-4. doi: 10.1111/j.1768-322x.1987.tb00519.x.

DOI:10.1111/j.1768-322x.1987.tb00519.x
PMID:3038233
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.

摘要

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