Ceramide Trihexosides (Globotriaosylceramides)

Ceramide trihexoside (globotriaosylceramide, Gb₃, or 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 In order to show affinity in binding, some toxins, such as veratoxins from Escherichia coli, require specific fatty acids on the ceramide portion of CTH. An accumulation of CTH in the cellular membranes due to a lack of alpha-galactosidase to convert it into lactosylceramide results in Fabry disease.² An inability to convert CTH to globoside due to mutations in the gene sequence leads to the P^k blood group phenotype. Under certain conditions CTH can enhance anticoagulant activity and has also been studied as a tool to investigate lymphocyte activation.³

References:

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., Vol. 27(6) pp. 1145-1150, 1989
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. 289–97, 2006
C. Menge, I. Stamm, M. Wuhrer, R. Geyer, L. H. Wieler, G. Baljer. “Globotriaosylceramide (Gb₃/CD77) is synthesized and surface expressed by bovine lymphocytes upon activation in vitro.” Vet Immunol Immunopathol., Vol. 83(1-2) pp. 19-36, 2001