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N-Glycinated lyso-ceramide trihexoside

CATALOG # 1530


  • Catalog #:1530
  • Scientific Name:N-Glycinated lyso-ceramide trihexoside
  • Common Name:N-Glycinated globotriaosylsphingosine
  • Empirical Formula:C38H70N2O18
  • CAS#:1360882-59-2
  • SDS:View Safety Data Sheet
  • Data Sheet:View Data Sheet
  • Formula Weight:843
  • Unit:1 mg
  • Solvent:none
  • Source:semisynthetic
  • Purity:98+%
  • Analytical Methods:TLC; identity confirmed by MS
  • Natural Source:porcine RBC
  • Solubility:chloroform/methanol/DI water, 2:1:0.1; DMF
  • Physical Appearance:solid
  • Storage:-20°C
  • Dry Ice:No
  • Hazardous:No


Application Notes:

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.

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
Price $624.00

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