N-Glycinated lyso-ceramide trihexoside

CATALOG # 1530
Amount 1 mg
Price $525.00
Qty
 
N-Glycinated lyso-ceramide trihexoside
  • Catalog #:1530
  • Scientific Name:N-Glycinated lyso-ceramide trihexoside
  • Common Name:N-Glycinated globotriaosylsphingosine
  • Empirical Formula:C38H70N2O18
  • SDSView 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
  • Literature References: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.

    References:
    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