Summary
Human umbilical venous endothelial cells were transformed with a temperature-sensitive mutant of simian virus 40, tsA640, and a cell line, subcultured for over 20 serial passages, was established at a temperature permissive for the virus. Treatment of transformed endothelium with 3μg/ml chloroquine caused a specific reduction of α-glactosidase activity, without cell injury, and revealed several electron-dense materials surrounded by single unit membranes. Crystalline lamellae in lysosomes with a periodicity of 6.5 nm, which are typically seen in various tissues in Fabry disease, were produced in the presence of a glycosphingolipid mixture. These cells should be useful for in vitro pathophysiological studies on Fabry endothelium.
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Supported in part by a Grant (3A-3) for Nervous and Mental Disorders from the Ministry of Health and Welfare, Japan
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Inagaki, M., Katsumoto, T., Nanba, E. et al. Lysosomal glycosphingolipid storage in chloroquine-induced α-galactosidase-deficient human endothelial cells with transformation by simian virus 40: in vitro model of Fabry disease. Acta Neuropathol 85, 272–279 (1993). https://doi.org/10.1007/BF00227722
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DOI: https://doi.org/10.1007/BF00227722