Defects in degradation of blood group A and B glycosphingolipids in Schindler and Fabry diseases

Defects in degradation of blood group A and B glycosphingolipids in Schindler and Fabry diseases

Skin fibroblast cultures from patients with inherited lysosomal enzymopathies, α-N-acetylgalactosaminidase (α-NAGA) and α-galactosidase A deficiencies (Schindler and Fabry disease, respectively), and from normal controls were used to study in situ degradation of blood group A and B glycosphingolipid...

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Bibliographic Details
Main Authors: Befekadu Asfaw, Jana Ledvinová, Robert Dobrovolńy, Henk D. Bakker, Robert J. Desnick, Otto P. van Diggelen, Jan G.N. de Jong, Tamotsu Kanzaki, Amparo Chabas, Irene Maire, Ernst Conzelmann, Detlev Schindler
Format: Article
Language:English
Published: Elsevier 2002-07-01
Series:Journal of Lipid Research
Subjects:
α-N-acetylgalactosaminidase deficiency
α-galactosidase A deficiency
skin fibroblasts
in situ metabolism
lysosome targeting
blood group glycolipids
Online Access:http://www.sciencedirect.com/science/article/pii/S0022227520314723

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http://www.sciencedirect.com/science/article/pii/S0022227520314723

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