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Biochimica Et Biophysica Acta|November 9, 2000
Quantification of mRNAs encoding proteins of the glycosphingolipid catabolism in mouse models of GM2 gangliosidoses and sphingolipid activator protein precursor (prosaposin) deficiencyA Potratz, S Hüttler, U Bierfreund, et al.
Proceedings of the National Academy of Sciences of the United States of America|August 4, 1999
A vital role for glycosphingolipid synthesis during development and differentiationT Yamashita, R Wada, T Sasaki, et al.
Biochimica Et Biophysica Acta|June 6, 2000
Characterization of two alpha-galactosidase mutants (Q279E and R301Q) found in an atypical variant of Fabry diseaseR Kase, U Bierfreund, A Klein, et al.
FEBS Letters|September 9, 1996
Only sphingolipid activator protein B (SAP-B or saposin B) stimulates the degradation of globotriaosylceramide by recombinant human lysosomal alpha-galactosidase in a detergent-free liposomal systemR Kase, U Bierfreund, A Klein, et al.
Neurochemical Research|February 11, 1999
Recombinant GM2-activator protein stimulates in vivo degradation of GA2 in GM2 gangliosidosis AB variant fibroblasts but exhibits no detectable binding of GA2 in an in vitro assayU Bierfreund, T Lemm, A Hoffmann, et al.
The Journal of Biological Chemistry|January 3, 2001
Mice expressing only monosialoganglioside GM3 exhibit lethal audiogenic seizuresH Kawai, M L Allende, R Wada, et al.
The Journal of Clinical Investigation|February 18, 1999
A genetic model of substrate deprivation therapy for a glycosphingolipid storage disorderY Liu, R Wada, H Kawai, et al.
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