Application Notes:
Ceramide trihexoside 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 Microbiol, Vol. 27:6 pp. 1145-1150, 1989
2. S. Bekri et al., Cardiovasc Hematol Agents Med Chem, Vol. 4:4 pp. 289–297, 2006
3. J. Groener et al., Clin Chem., Vol. 53:4 pp.742-747, 2007
4. K. Mills, A. Johnson, B. Winchester, FEBS Lett., Vol. 515 pp. 171-176, 2002
5. C. Menge et al., Vet Immunol Immunopathol., Vol. 83 pp.19-36, 2001