N-Glycinated lyso-ceramide trihexoside/N-甘氨酸溶血神经酰胺三己糖苷/1360882-59-2
Specifications
Catalog #:1530
Scientific Name:N-Glycinated lyso-ceramide trihexoside
Common Name:N-Glycinated globotriaosylsphingosine
Empirical Formula:C38H70N2O18
CAS#:1360882-59-2
SDS:View Safety Data Sheet
Data Sheet:View Data Sheet
Formula Weight:843
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; DMF
Physical Appearance:solid
Storage:-20℃
Dry Ice:No
Hazardous:No
Description
Application Notes:
n -甘氨酸化的溶神经酰胺三己糖是一种重要的生物分子溶神经酰胺三己糖(globotriaosylsphingosine, lyso-Gb3)的类似物。理想作为内部标准的提取和质谱(MS)分析lyso-ceramide trihexoside。(1)自由胺组给这个产品非常相似的物理特性对自然lyso-ceramide trihexoside而甘氨酸增加了一个额外的57个单位女士的分子便于检测。(2)法布里病的特征是缺乏-半乳糖苷酶(-半乳糖苷酶)。(3)早期发现和治疗是防止各种器官损伤的关键。
N-Glycinated lyso-ceramide trihexoside is an analogue of the important biomolecule lyso-ceramide trihexoside (globotriaosylsphingosine, lyso-Gb3). It is ideal for use as an internal standard in the extraction and mass spectrometry (MS) analysis of lyso-ceramide trihexoside.(1) The free amine group gives this product very similar physical characteristics to the natural lyso-ceramide trihexoside while the glycine adds an additional 57 units to the molecule making it easy to detect by MS. lyso-Ceramide trihexoside and the acylated ceramide trihexoside (globotriaosylceramide, Gb3) are important biomarkers for the lysosomal storage disorder Fabry disease.(2) Fabry disease is characterized by a deficiency in the enzyme - galactosidase, resulting in an accumulation of ceramide trihexoside and lyso-ceramide trihexoside.(3) Early detection and treatment of this disease is critical to prevent damage to various organs.
References:
1. R. Krüger et al. Quantification of the Fabry marker lysoGb3 in human plasma by tandem mass spectrometry. Journal of Chromatography B., Vol. 883-884, pp. 128-135, 2012
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., Vol. 4:4 pp. 289–297, 2006
3. C. Auray-Blais et al. How well does urinary lyso-Gb3 function as a biomarker in Fabry disease. Clin. Chim. Acta, Vol. 411(23-24) pp. 1906-1914, 2010
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