Lyso-Ceramide trihexoside contains a free amine linkage enabling well-defined ceramide trihexosides to be produced. Ceramide trihexoside 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 membrane due to a lack of alpha-galactosidase to convert it into lactosyl ceramide results in Fabry disease.2 It can be used as an excellent standard for the identification of CTH in Fabry disease by HPLC3 and mass spectrometry.4 In contrast to Fabry disease, a deficiency of CTH 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
