N-Heptadecanoyl-ceramide trihexoside/N-十七烷酰基-神经酰胺三己糖苷/536745-81-0
Specifications
Catalog #:1523
Scientific Name:N-Heptadecanoyl-ceramide trihexoside
Common Name:N-C17:0-Ceramide trihexoside; N-Heptadecanoyl globotriaosylceramide
Empirical Formula:C53H99NO18
CAS#:536745-81-0
SDS:View Safety Data Sheet
Data Sheet:View Data Sheet
Formula Weight:1038
Unit:500 µg
Solvent:none
Source:semisynthetic
Purity:98+%
Analytical Methods:TLC, HPLC; identity confirmed by MS
Natural Source:porcine RBC
Solubility:DMSO, hot methanol, chloroform/methanol, 2:1
Physical Appearance:solid
Storage:-20℃
Dry Ice:No
Hazardous:No
Description
Application Notes:
该产品是一种定义明确的CTH，含有罕见的十七酸酰化成神经酰胺，使其成为理想的内标和生物学研究神经酰胺三己糖苷(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，并已被确定为各种毒素的受体，包括志贺毒素和志贺样毒素一些毒素，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病本品可作为法布瑞氏病中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 product is a well-defined CTH containing the uncommon heptadecanoic acid acylated to the ceramide making it ideal as an internal standard and for biological studies.1 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.2 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.3 This product can be used as an excellent standard for the identification of CTH in Fabry disease by HPLC4 and mass spectrometry. 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. M. Fuller et al. “Urinary Lipid Profiling for the Identification of Fabry Hemizygotes and Heterozygotes” Clinical Chemistry, Vol. 51 pp. 688-694, 2005
2. 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
3. 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
4. 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
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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