Application Notes:
Ceramide trihexoside (CTH) 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.1 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.2 This product can be used as an excellent standard for the identification of CTH in Fabry
disease by HPLC3 and mass spectrometry.4 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. S. Ashkenazi and T. G. Cleary, J Clin Microbio. June; 27(6): 1145-1150, 1989
2. S. Bekri, O. Lidove, R. Jaussaud, B. Knebelmann, F. Barbey. Cardiovasc Hematol Agents Med Chem 4 (4): 289–97, October 2006
3. J. E. Groener, B. J. Poorthuis, S. Kuiper, M. T. Helmond, C. E. Hollak, J. M. Aerts. Clin Chem., Apr;53(4):742-7, 2007. Epub Mar 1 2007
4. K. Mills, A. Johnson, B. Winchester. FEBS Lett., Mar 27;515(1-3):171-6, 2002
5. C. Menge, I. Stamm, M. Wuhrer, R. Geyer, L. H. Wieler, G. Baljer., Vet Immunol Immunopathol., Nov;83(1-2):19-36, 2001