Ceramide trihexosides, (bottom spot)Ceramide trihexosides, (bottom spot)
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Ceramide trihexosides, (bottom spot)

CTH with hydroxy fatty acid side chain

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 Neutral glycolipids may help to cause resistance to natural killer cells in some types of cells. Interferon appears to slightly increase the resistance of cells towards natural killer cells and also increases the amounts of non-hydroxylated fatty acids on various glycolipids, suggesting that there may be a connection between the hydroxylated fatty acids and natural killer cell resistance.
Cat# Size Price Qty Buy
1514 0.5 mg £328.13

Additional Information

Property Value or Rating
Product Size 0.5 mg
Manufacturer Matreya, LLC
Empirical Formula C60H113NO19 (2-hydroxytetracosanoyl)
Formula Weight 1152.5
Solvent none
Source natural
Purity 98+%
Analytical Methods TLC; identity confirmed by MS
Natural Source Animal/Porcine RBC
Solubility hot methanol, DMSO, chloroform/methanol, 1:1
Physical Appearance A neat solid
Storage -20°C
References

1. 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 
2. 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 
3. 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 
4. K. Mills, A. Johnson, B. Winchester. “Synthesis of novel internal standards for the quantitative determination of plasma ceramide trihexoside in Fabry disease by tandem mass spectrometry.” FEBS Lett., Mar 27;515(1-3):171-6, 2002 
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

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