In vitro inhibition and intracellular enhancement of lysosomal α‐galactosidase A activity in Fabry lymphoblasts by 1‐deoxygalactonojirimycin and its derivatives

Asano, Naoki; Ishii, Satoshi; Kizu, Haruhisa; Ikeda, Kyoko; Yasuda, Kayo; Kato, Atsushi; R. Martin, Olivier; Fan, Jianqiang

doi:10.1046/j.1432-1327.2000.01457.x

Public

In vitro inhibition and intracellular enhancement of lysosomal α‐galactosidase A activity in Fabry lymphoblasts by 1‐deoxygalactonojirimycin and its derivatives

Published in European Journal of Biochemistry • Jul 1, 2000

Authors:

Naoki Asano

Satoshi Ishii

Haruhisa Kizu

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...

Subject

Intracellular

Lymphoblast

Alpha-galactosidase

Finding related papers...

Discussions

(0)

No comments yet

Be the first to share your thoughts!