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About This Item
Empirical Formula (Hill Notation):
C24H42D5NO7
CAS Number:
Molecular Weight:
466.66
UNSPSC Code:
12352211
NACRES:
NA.12
Product Name
Galactosyl(β) Sphingosine-d5, Avanti Research™ - A Croda Brand 860637P, powder
InChI key
HHJTWTPUPVQKNA-LTNFHSIGSA-N
SMILES string
[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
assay
>99% (TLC)
form
powder
packaging
pkg of 1 × 1 mg (860637P-1mg)
manufacturer/tradename
Avanti Research™ - A Croda Brand 860637P
shipped in
dry ice
storage temp.
−20°C
Application
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.
Biochem/physiol Actions
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.
Packaging
5 mL Amber Glass Screw Cap Vial (860637P-1mg)
Legal Information
Avanti Research is a trademark of Avanti Polar Lipids, LLC
Storage Class
11 - Combustible Solids
flash_point_f
No data available
flash_point_c
No data available
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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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