Product Information
- LysoglobotriaosylceramidelysoGb3
- Α-D-Gal-(1→4)-Β-D-Gal-(1→4)-Β-D-Glc-1→O-Sphingosine
- Globotriaosyl Lysosphingolipid
- Globotriaosylsphingosine from porcine blood
- (2S,3R,4E)-2-amino-3-hydroxyoctadec-4-en-1-yl alpha-D-galactopyranosyl-(1->4)-beta-D-galactopyranosyl-(1->4)-beta-D-glucopyranoside
Globotriaosylsphingosine is dramatically increased in plasma of classically affected male Fabry patients and plasma and tissues of Fabry mice. Globotriaosylsphingosine was shown to be an inhibitor of α-galactosidase A activity. Furthermore, exposure of smooth muscle cells, but not fibroblasts, to globotriaosylsphingosine at concentrations observed in plasma of patients promotes proliferation. It was suggested that measurement of circulating globotriaosylsphingosine would be useful to monitor Fabry disease and may contribute to a better understanding of the disorder.
Chemical properties
Technical inquiry about: 3D-OG44865 Globotriaosylsphingosine
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