Application Notes:
This product is a well-defined ceramide trihexoside containing a hexadecanoic acid acylated to the ceramide moiety making
it ideal as an internal standard and for biological studies.1 Ceramide trihexoside (CTH or Gb3) is a glycosphingolipid found
mostly in mammalian cell membranes. It is involved in cellular signaling and has been identified as a receptor for various
toxins including shiga toxins and shiga-like toxins.2 Some toxins, such as veratoxins from Escherichia coli, require specific
fatty acids on the ceramide portion of CTH to show affinity in binding. An accumulation of CTH in the cellular membranes
due to a lack of alpha-galactosidase to convert it into lactosyl ceramide results in Fabry disease.3 This product can be used as
an excellent standard for the identification of CTH in Fabry disease by HPLC4 and mass spectrometry. An inability to convert
CTH to globoside due to mutations in the gene sequence leads to the Pk blood group phenotype. It appears that under certain
conditions CTH can enhance anticoagulant activity. CTH has also been studied as a tool to investigate lymphocyte
activation.5
References:
1. M. Fuller et al. “Urinary Lipid Profiling for the Identification of Fabry Hemizygotes and Heterozygotes” Clinical Chemistry, Vol. 51 pp. 688-694, 2005
2. S. Ashkenazi and T. G. Cleary, “Rapid method to detect shiga toxin and shiga-like toxin I based on binding to globotriosyl ceramide (Gb3), their natural
receptor.” J Clin Microbio. June; 27(6): 1145-1150, 1989
3. 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 4 (4): 289–97, October 2006
4. J. E. Groener, B. J. Poorthuis, S. Kuiper, M. T. Helmond, C. E. Hollak, J. M. Aerts. “HPLC for simultaneous quantification of total ceramide,
glucosylceramide, and ceramide trihexoside concentrations in plasma.” Clin Chem., Apr;53(4):742-7, 2007. Epub Mar 1 2007
5. C. Menge, I. Stamm, M. Wuhrer, R. Geyer, L. H. Wieler, G. Baljer. “Globotriaosylceramide (Gb(3)/CD77) is synthesized and surface expressed by
bovine lymphocytes upon activation in vitro.” Vet Immunol Immunopathol., Nov;83(1-2):19-36, 2001