N-Glycinated lyso-ceramide trihexosideN-Glycinated lyso-ceramide trihexoside
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N-Glycinated lyso-ceramide trihexoside

N-Glycinated globotriaosylsphingosine

N-Glycinated lyso-ceramide trihexoside is an analogue of the important biomolecule lyso-ceramide trihexoside (globotriaosylsphingosine, lyso-Gb3). It is ideal for use as an internal standard in the extraction and mass spectrometry (MS) analysis of lyso-ceramide trihexoside.(1) The free amine group gives this product very similar physical characteristics to the natural lyso-ceramide trihexoside while the glycine adds an additional 57 units to the molecule making it easy to detect by MS. lyso-Ceramide trihexoside and the acylated ceramide trihexoside (globotriaosylceramide, Gb3) are important biomarkers for the lysosomal storage disorder Fabry disease.(2) Fabry disease is characterized by a deficiency in the enzyme - galactosidase, resulting in an accumulation of ceramide trihexoside and lyso-ceramide trihexoside.(3) Early detection and treatment of this disease is critical to prevent damage to various organs.
Cat# Size Price Qty Buy
1530 1 mg £469.20

Additional Information

Property Value or Rating
Product Size 1 mg
Manufacturer Matreya, LLC
Empirical Formula C38H70N2O18
CAS# 1360882-59-2
Formula Weight 843
Solvent none
Source semisynthetic
Purity 98+%
Analytical Methods TLC; identity confirmed by MS
Natural Source Animal/Porcine RBC|Semi-synthetic
Solubility chloroform/methanol/DI water, 2:1:0.1; DMF
Physical Appearance A neat solid
Storage -20°C

1. R. Krüger et al. Quantification of the Fabry marker lysoGb3 in human plasma by tandem mass spectrometry. Journal of Chromatography B., Vol. 883-884, pp. 128-135, 2012 
2. S. Bekri, O. Lidove, R. Jaussaud, B. Knebelmann, F. Barbey The role of ceramide trihexoside (globotriaosylceramide) in the diagnosis and follow-up of the efficacy of treatment of Fabry disease: a review of the literature. Cardiovasc Hematol Agents. Med. Chem., Vol. 4:4 pp. 289–297, 2006 
3. C. Auray-Blais et al. How well does urinary lyso-Gb3 function as a biomarker in Fabry disease. Clin. Chim. Acta, Vol. 411(23-24) pp. 1906-1914, 2010

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