| 包装: | 500ug |
| 规格: | 98% |
| 市场价: | 7334元 |
| 分子量: | 1055.4 |
Background:
C18 Globotriaosylceramide-d3is 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[Matreya, LLC. Catalog No. 1537]
1.Kamani, M.A., ProvenÇal, P., Boutin, M., et al.Glycosphingolipid storage in Fabry mice extends beyond globotriaosylceramide and is affected by ABCB1 depletionFuture Sci. OA.2(4)FS0147(2016)
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