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表单
powder
包装
pkg of 1 × 1 mg (860637P-1mg)
制造商/商品名称
Avanti Research™ - A Croda Brand 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
InChI key
HHJTWTPUPVQKNA-LTNFHSIGSA-N
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)
法律信息
Avanti Research is a trademark of Avanti Polar Lipids, LLC
储存分类代码
11 - Combustible Solids
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
Catherine O'Sullivan et al.
Journal of cell science, 128(21), 3878-3887 (2015-09-12)
Globoid cell leukodystrophy (Krabbe disease) is a rare infantile neurodegenerative disorder. Krabbe disease is caused by deficiency in the lysosomal enzyme galactocerebrosidase (GALC) resulting in accumulation, in the micromolar range, of the toxic metabolite galactosylsphingosine (psychosine) in the brain. Here
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