C18 Globotriaosylceramide-d3 (d18:1/18:0-d3)

C18 Globotriaosylceramide-d3 is intended for use as an internal standard for the quantification of C18 globotriaosylceramide by GC- or LC-MS. C18 globotriaosylceramide is an endogenous sphingolipid that accumulates in the brain, heart, kidney, liver, lung, and spleen in a mouse model of Fabry disease, a lysosomal storage disorder characterized by a deficiency in the enzyme α-galactosidase A.1 As this product is derived from a natural source, there may be variations in the sphingoid backbone. [Matreya, LLC. Catalog No. 1537]WARNING This product is not for human or veterinary use.