Lysosomal Glycosphingolipid Storage in Chloroquine-induced Alpha-galactosidase-deficient Human Endothelial Cells with Transformation by Simian Virus 40: in Vitro Model of Fabry Disease

Overview

Journal Acta Neuropathol

Specialty Neurology

Date 1993 Jan 1

PMID 8384772

Citations 5

Authors

M Inagaki

T Katsumoto

E Nanba

K Ohno

S Suehiro

K Takeshita

Affiliations

Soon will be listed here.

Abstract

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 3 micrograms/ml chloroquine caused a specific reduction of alpha-galactosidase 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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