Ceramide trihexosides (bottom spot)/神经酰胺三己糖苷 (底点)

Specifications

• Catalog #:1514

• Scientific Name:Ceramide trihexosides (bottom spot)

• Common Name:CTH with hydroxy fatty acid side chain

• Empirical Formula:C₆₀H₁₁₃NO₁₉ (2-hydroxytetracosanoyl)
  Typical fatty acid content

• SDS:View Safety Data Sheet

• Data Sheet:View Data Sheet

• Formula Weight:1153 (2-hydroxytetracosanoyl)

• Unit:500 ug

• Solvent:none

• Source:natural

• Purity:98+%

• Analytical Methods:TLC; identity confirmed by MS

• Natural Source:porcine RBC

• Solubility:hot methanol, DMSO, chloroform/methanol, 1:1

• 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中性糖脂可能有助于在某些类型的细胞中引起对自然杀伤细胞的抵抗。干扰素似乎能轻微增加细胞对自然杀伤细胞的抵抗力，也能增加各种糖脂上非羟基化脂肪酸的数量，提示羟基化脂肪酸与自然杀伤细胞抗性之间可能存在联系。

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.

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