N-Glycinated lyso-ceramide trihexoside

Catalogue number:

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1530-1 mg

Supplier:

Matreya

Size:

1 mg

N/A

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CAS Number:

1360882-59-2

Purity:

≥98%

Formulation:

A neat solid

Molecular Formula:

C38H70N2O18

Molecular Weight:

843

Alternative Names:

N-Glycinated globotriaosylsphingosine

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Product Description:

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 _x005F_x0001_- 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.