C18 3'-sulfo Galactosylceramide-d3 is intended for use as an internal standard for the quantification of C18 3'-sulfo galactosylceramide by GC- or LC-MS. C18 3'-sulfo Galactosylceramide is a minor endogenous sphingolipid produced from C18 ceramide and UDP-galactose in the endoplasmic reticulum followed by sulfation in the Golgi apparatus.1 It is found in pro-oligodendroblasts and in neurons and accumulates in the lysosome of patients with metachromatic leukodystrophy, a disorder characterized by arylsulfatase A deficiency.2,1,3 C18 3'-sulfo Galactosylceramide also increases expression of indoleamine 2,3-dioxygenase 1 (DOX1) to catalyze the initial and rate-limiting step in kynurenine pathway tryptophan catabolism in antigen presenting cells to regulate several immune responses.1 [Matreya, LLC. Catalog No. 1536]
1.Takahashi, T., and Suzuki, T.Role of sulfatide in normal and pathological cells and tissuesJ. Lipid Res.53(8)1437-1450(2012) 2.Saville, J.T., Smith, N.J.C., Fletcher, J.M., et al.Quantification of plasma sulfatides by mass spectrometry: Utility for metachromatic leukodystrophyAnal. Chim. Acta95579-85(2017) 3.Svennerholm, L., and StÄllberg-Stenhagen, S.Changes in the fatty acid composition of cerebrosides and sulfatides of human nervous tissue with ageJ. Lipid Res.9(2)215-225(1968)