lyso-Ceramide trihexoside/溶血神经酰胺三己糖苷/126550-86-5
Specifications
Catalog #:1520
Scientific Name:lyso-Ceramide trihexoside
Common Name:lyso-CTH; lyso-Globotriaosylsphingosine
Empirical Formula:C36H67NO17
CAS#:126550-86-5
SDS:View Safety Data Sheet
Data Sheet:View Data Sheet
Formula Weight:786
Unit:1 mg
Solvent:none
Source:semisynthetic
Purity:98+%
Analytical Methods:TLC; identity confirmed by MS
Natural Source:porcine RBC
Solubility:chloroform/methanol/DI water, 2:1:0.1; DMSO
Physical Appearance:solid
Storage:-20℃
Dry Ice:No
Hazardous:No
Description
Application Notes:
Lyso-Ceramide trihex糖苷含有一个自由的胺键，可以产生良好定义的神经酰胺三己糖。神经酰胺三己糖苷参与细胞信号转导，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与Fabry病相反，基因序列突变导致的CTH缺失导致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。
Lyso-Ceramide trihexoside contains a free amine linkage enabling well-defined ceramide trihexosides to be produced. Ceramide trihexoside 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 membrane due to a lack of alpha-galactosidase to convert it into lactosyl ceramide results in Fabry disease.2 It can be used as an excellent standard for the identification of CTH in Fabry disease by HPLC3 and mass spectrometry.4 In contrast to Fabry disease, a deficiency of CTH 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. Jacewicz, H. Clausen, E. Nudelman, A. Donohue-Rolfe, G. Keusch, J Exp Med., Vol.163:6 pp.1391-1404, 1986
2. S. Bekri, O. Lidove, R. Jaussaud, B. Knebelmann, F. Barbey, Med Chem Vol. 4:4 pp. 289–297, 2006
3. J. Groener, et al., Clin Chem. Vol. 53:4 pp.742-747, 2007
4. K. Mills, A. Johnson, B. Winchester, FEBS Lett., Vol. 515 pp. 171-176, 2002
5. C. Menge et al., Vet Immunol Immunopathol., Vol. 83 pp.19-36, 2001
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