In vitro inhibition and intracellular enhancement of lysosomal alpha-galactosidase A activity in Fabry lymphoblasts by 1-deoxygalactonojirimycin and its derivatives.

N, Asano; S, Ishii; H, Kizu; K, Ikeda; K, Yasuda; A, Kato; OR, Martin; JQ, Fan

doi:10.1046/j.1432-1327.2000.01457.x

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In vitro inhibition and intracellular enhancement of lysosomal alpha-galactosidase A activity in Fabry lymphoblasts by 1-deoxygalactonojirimycin and its derivatives.

Shared by NobleBlocks on Jul 1, 2000 • 12:00 AM UTC

Authors:

Asano N

Ishii S

Kizu H

Abstract

Fabry disease is a lysosomal storage disorder caused by deficient lysosomal alpha-galactosidase A (alpha-Gal A) activity. Deficiency of the enzyme activity results in progressive deposition of neutral glycosphingolipids with terminal alpha-galactosyl residue in vascular endothelial cells. We recentl...

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