Ceramide trihexosides; Gb3 (10 mg)/神经酰胺三己糖苷; Gb3 (10 mg)/71965-57-6
Specifications
Catalog #:1067-10
Scientific Name:Ceramide trihexosides
Common Name:CTH; Gb3; Globotriaosylceramide
Empirical Formula:C60H113NO18 (tetracosanoyl)
Typical fatty acid contentCAS#:71965-57-6
SDS:View Safety Data Sheet
Data Sheet:View Data Sheet
Formula Weight:1137 (tetracosanoyl)
Unit:10 mg
Solvent:none
Source:natural
Purity:98+%
Analytical Methods:TLC; identity confirmed by MS
Natural Source:porcine RBC
Solubility:chloroform/methanol, 2:1; DMSO, hot methanol
Physical Appearance:solid
Storage:-20℃
Dry Ice:No
Hazardous:No
Description
Application Notes:
神经酰胺三己糖苷(CTH)是一种主要存在于哺乳动物细胞膜上的鞘糖脂。它参与细胞信号转导，已被确定为各种毒素的受体，包括志贺毒素和志贺类毒素一些毒素，如来自大肠杆菌的维拉毒素，需要CTH神经酰胺部分的特定脂肪酸来显示亲和力结合。由于缺乏半乳糖苷酶将CTH转化为乳糖酰神经酰胺，CTH在细胞膜上积累，导致Fabry病本品可作为法布里病CTH的高效液相色谱和质谱鉴定的优良标准品由于基因序列突变，CTH无法转化为球形糖苷，导致Pk血型表型。在一定条件下，CTH可以增强抗凝血活性。CTH也被用作研究淋巴细胞活化的工具。
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
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