ISSN:
1432-0533
Keywords:
Fabry disease
;
Endothelial cells culture
;
Simian virus 40
;
Transformation
;
Chloroquine
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
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.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00227722
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