lactosylsphingosine (d-18:1)

Lactosylsphingosine is a bioactive sphingolipid and a form of lactosylceramide that is lacking the fatty acyl group. Lyso-lactosylceramide (1-50 μM) reduces viability of human neutrophils in a concentration-dependent manner. Unlike lactosylceramide,lactosylsphingosine has no effect on protein synthesis and cell proliferation in cardiomyocytes. Lactosylsphingosine is a precursor in the synthesis of lyso-ganglioside GM3.

Lyso-globotriaosylceramide is a form of globotriaosylceramide that is lacking the fatty acyl group. It binds to Shiga toxin 1 (Stx1) in the presence of cholesterol and phosphatidylcholine but does not bind Stx2. It also reduces viability and aggregation of human neutrophils induced by phorbol 12-myristate 13-acetate when used at concentrations of 50 and 1 μM, respectively. Lyso-globotriaosylceramide 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. It also accumulates in the urine, kidney, and plasma of patients with Fabry disease. Lyso-globotriaosylceramide levels decrease in response to administration of the α-galactosidase inhibitor 1-deoxygalactonojirimycin in a transgenic mouse model of Fabry disease. Decreases in plasma and urine concentrations of lyso-globotriaosylceramide have been used as a biomarker for efficacy of enzyme replacement therapy (ERT) and other therapies in the treatment of Fabry disease.

Sphingomyelins are complex membrane lipids composed of phosphorylcholine, sphingosine, and an acylated group, such as a fatty acid. Lysosphingomyelin is a naturally-occurring lipid which is produced by the removal of the acylated group of sphingomyelin by a deacylase. Lysosphingomyelin may, in turn, serve as a substrate for autotaxin, which removes choline to produce sphingosine-1-phosphate. The receptors and signaling pathways that are activated by lyso-sphingosine are diverse and vary between cell types. Lysosphingomyelin occurs naturally in plasma, is a constituent of lipoproteins, and is increased in some diseases, including dermatitis and Niemann-Pick disease.

13C6 Glucosylsphingosine (d18:1) is an isotopically enriched form of 1-β-D-glucosylsphingosine (d18:1) that is intended for use as an internal standard for the quantification of 1-β-D-glucosylsphingosine by GC- or LC-MS. 1-β-D-Glucosylsphingosine is a lysolipid derivative of glucosylcerebroside that decreases activity of glucocerebrosidase in LA-N-2 cells in a dose-dependent manner.

L-threo Lysosphingomyelin (d18:1) (L-threo-Sphingosylphosphorylcholine) is a naturally occurring bioactive sphingolipid that functions as a potent agonist for S1P receptors, with EC50 values of 19.3, 131.8, and 313.3 nM for hS1P1, hS1P3, and hS1P2, respectively.

Lysosphingomyelin is an endogenous bioactive sphingolipid and a constituent of lipoproteins.1,2It is produced by the removal of the acyl group from sphingomyelin by a deacylase and acts as a precursor in the biosynthesis of sphingosine-1-phosphate . D-erythroLysosphingomyelin is an agonist of the S1P receptors S1P1, S1P2, and S1P3(EC50s = 167.7, 368.1, and 482.6 nM, respectively, for the human receptors).3It is also an agonist of the orphan receptor ovarian cancer G protein-coupled receptor 1 (ORG1) that induces calcium accumulation in cells overexpressing OGR1 (EC50= ~35 nM).4Levels of D-erythrolysosphingomyelin are increased in skin isolated from patients with atopic dermatitis, as well as postmortem brain from patients with Niemann-Pick disease type A, but not type B.2,5L-threolysosphingomyelin is also an S1P1-3agonist (EC50s = 19.3, 131.8, and 313.3 nM, respectively).3This product is a mixture of D-erythroand L-threolysosphingomyelin. [Matreya, LLC. Catalog No. 1321] 1.Ito, M., Kurita, T., and Kita, K.A novel enzyme that cleaves the N-acyl linkage of ceramides in various glycosphingolipids as well as sphingomyelin to produce their lyso formsJ. Biol. Chem.270(41)24370-24374(1995) 2.Nixon, G.F., Mathieson, F.A., and Hunter, I.The multi-functional role of sphingosylphosphorylcholineProg. Lipid Res.47(1)62-75(2008) 3.Im, D.-S., Clemens, J., Macdonald, T.L., et al.Characterization of the human and mouse sphingosine 1-phosphate receptor, S1P5 (Edg-8): Structure-activity relationship of sphingosine1-phosphate receptorsBiochemistry40(46)14053-14060(2001) 4.Meyer zu Heringdorf, D., Himmel, H.M., and Jakobs, K.H.Sphingosylphosphorylcholine-biological functions and mechanisms of actionBiochim. Biophys. Acta1582(1-3)178-189(2002) 5.Rodriguez-Lafrasse, C., and Vanier, M.T.Sphingosylphosphorylcholine in Niemann-Pick disease brain: Accumulation in type A but not in type BNeurochem. Res.24(2)199-205(1999)