推荐产品
形式
powder
包装
pkg of 1 × 1 mg (860637P-1mg)
制造商/商品名称
Avanti Polar Lipids 860637P
运输
dry ice
储存温度
−20°C
SMILES字符串
[H][C@](/C=C/CCCCCCCCCCCC([2H])(C([2H])([2H])[2H])[2H])(O)[C@](N)([H])CO[C@H](O1)[C@H](O)[C@@H](O)[C@H]([C@H]1CO)O
应用
Galactosyl(β) Sphingosine-d5 has been used as an internal standard for the quantification of galactosylsphingosine, in the extracts from the mouse cerebellum by liquid chromatography-tandem mass spectrometry.
生化/生理作用
Galactosylsphingosine, also known as psychosine, has an ability to induce apoptosis in various types of cells including oligodendrocytes. It is a toxic metabolite. Accumulation of this cytotoxic sphingolipid in the brain due to the deficiency of galactocerebrosidase (GALC) leads to the development of Krabbe disease.
包装
5 mL Amber Glass Screw Cap Vial (860637P-1mg)
闪点(°F)
No data available
闪点(°C)
No data available
Jacqueline A Hawkins-Salsbury et al.
Journal of lipid research, 54(12), 3303-3311 (2013-09-06)
Globoid cell leukodystrophy (GLD) is a neurological disease caused by deficiency of the lysosomal enzyme galactosylceramidase (GALC). In the absence of GALC, the cytotoxic glycosphingolipid, psychosine (psy), accumulates in the nervous system. Psychosine accumulation preferentially affects oligodendrocytes, leading to progressive
Victoria Schiffer et al.
PloS one, 15(1), e0227077-e0227077 (2020-01-14)
Gaucher disease is caused by a deficiency in glucocerebrosidase that can result in non-neuronal as well as neuronal symptoms. Common visceral symptoms are an increased organ size, specifically of the spleen, and glucosylceramide as well as glucosylsphingosine substrate accumulations as
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