A globotriaosylceramide lacking the fatty acyl group. 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 alpha-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. Synonyms: a-D-Gal-(14)-b-D-Gal-(14)-b-D-Glc-1O-sphingosine, Lysoglobotriaosylceramide, lyso-Ceramide Trihexoside, beta-D-Glucopyranoside, (2S,3R,4E)-2-amino-3-hydroxy-4-octadecen-1-yl O-alpha-D-galactopyranosyl-(14)-O-beta-D-galactopyranosyl-(14)- CAS No: 126550-86-5 Molecular Formula: C36H67NO17 Molecular Weight: 785.91 Appearance: White to off-white powder Purity: 98% Solubility: Chloroform:Methanol:Water (4:3:1) Storage and Stability: May be stored at RT for short-term only. Long-term storage is recommended at -20C. For maximum recovery of product, centrifuge the original vial prior to removing the cap.
Molecular Weight:
785.91
Purity:
98%
Form:
White to off-white powder
CAS Number:
[126550-86-5]
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