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Results: 1-7 |
Results: 7
Mice expressing only monosialoganglioside GM3 exhibit lethal audiogenic seizures
Authors: Kawai, H Allende, ML Wada, R Kono, M Sango, K Deng, CX Miyakawa, T Crawley, JN Werth, N Bierfreund, U Sandhoff, K Proia, RL
Citation: H. Kawai et al., Mice expressing only monosialoganglioside GM3 exhibit lethal audiogenic seizures, J BIOL CHEM, 276(10), 2001, pp. 6885-6888
Quantification of mRNAs encoding proteins of the glycosphingolipid catabolism in mouse models of GM2 gangliosidoses and sphingolipid activator protein precursor (prosaposin) deficiency
Authors: Potratz, A Huttler, S Bierfreund, U Proia, RL Suzuki, K Sandhoff, K
Citation: A. Potratz et al., Quantification of mRNAs encoding proteins of the glycosphingolipid catabolism in mouse models of GM2 gangliosidoses and sphingolipid activator protein precursor (prosaposin) deficiency, BBA-MOL BAS, 1502(3), 2000, pp. 391-397
Characterization of two alpha-galactosidase mutants (Q279E and R301Q) found in an atypical variant of Fabry disease
Authors: Kase, R Bierfreund, U Klein, A Kolter, T Utsumi, K Itoh, K Sandhoff, K Sakuraba, H
Citation: R. Kase et al., Characterization of two alpha-galactosidase mutants (Q279E and R301Q) found in an atypical variant of Fabry disease, BBA-MOL BAS, 1501(2-3), 2000, pp. 227-235
Sphingolipid hydrolases and activator proteins
Authors: Bierfreund, U Kolter, T Sandhoff, K
Citation: U. Bierfreund et al., Sphingolipid hydrolases and activator proteins, METH ENZYM, 311, 2000, pp. 255-276
Recombinant GM2-activator protein stimulates in vivo degradation of GA2 inGM2 gangliosidosis AB variant fibroblasts but exhibits no detectable binding of GA2 in an in vitro assay
Authors: Bierfreund, U Lemm, T Hoffmann, A Uhlhorn-Dierks, G Childs, RA Yuen, CT Feizi, T Sandhoff, K
Citation: U. Bierfreund et al., Recombinant GM2-activator protein stimulates in vivo degradation of GA2 inGM2 gangliosidosis AB variant fibroblasts but exhibits no detectable binding of GA2 in an in vitro assay, NEUROCHEM R, 24(2), 1999, pp. 295-300
A vital role for glycosphingolipid synthesis during development and differentiation
Authors: Yamashita, T Wada, R Sasaki, T Deng, CX Bierfreund, U Sandhoff, K Proia, RL
Citation: T. Yamashita et al., A vital role for glycosphingolipid synthesis during development and differentiation, P NAS US, 96(16), 1999, pp. 9142-9147
A genetic model of substrate deprivation therapy for a glycosphingolipid storage disorder
Authors: Liu, YJ Wada, R Kawai, H Sango, K Deng, CX Tai, T McDonald, MP Araujo, K Crawley, JN Bierfreund, U Sandhoff, K Suzuki, K Proia, RL
Citation: Yj. Liu et al., A genetic model of substrate deprivation therapy for a glycosphingolipid storage disorder, J CLIN INV, 103(4), 1999, pp. 497-505
Risultati: 1-7 |