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N-Hexadecanoyl-D9 (13,13,14,14,15,15,16,16,16)-ceramide trihexoside/N-十六烷酰基-D9 (13,13,14,14,15,15,16,16,16)-神经酰胺三己糖苷

Specifications

  • Catalog #:1551

  • Scientific Name:N-Hexadecanoyl-D9 (13,13,14,14,15,15,16,16,16)-ceramide trihexoside

  • Common Name:CTH-D9 ; Gb3-D9 ; Globotriaosylceramide-D9

  • Empirical Formula:C52H88D9NO18

  • SDS:View Safety Data Sheet

  • Data Sheet:View Data Sheet

  • Formula Weight:1033

  • Unit:500 µg

  • Source:semisynthetic, porcine RBC

  • Purity:98+%

  • Analytical Methods:TLC; identity confirmed by MS

  • Solubility:Chloroform/methanol/DI water, 2:1:0.1

  • Physical Appearance:solid

  • Storage:-20°C

  • Dry Ice:No

  • Hazardous:No

Description

Application Notes:

这种高纯度氘化产品是理想的鉴定神经酰胺三己糖苷样品和生物系统。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。

This high purity deuterated product is ideal for the identification of ceramide trihexoside in samples and biological systems. 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, “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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