C18 globotriaosylceramide is an endogenous sphingolipid found in mammalian cell membranes that is synthesized from lactosylceramide . It inhibits aggregation of human neutrophils induced by phorbol 12-myristate 13-acetate (PMA; 10008014) when used at a concentration of 1 μM. C18 globotriaosylceramide acts as a receptor for Shiga toxin in B cell-derived Raji cells and THP-1 monocytes. It 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. C18 globotriaosylceramide also accumulates in endothelial cells, pericytes, vascular smooth muscle cells, renal epithelial cells, dorsal ganglia neuronal cells, and myocardial cells in patients with Fabry disease.

C2 Adamantanyl Galactosylceramide (d18:1/2:0) (AdaGalCer) is a bioactive sphingolipid. It reduces globotriaosylceramide 3 synthesis from exogenous lactosylceramide in microsomes. AdaGalCer stimulates recombinant glucocerebrosidase activity in a pH-dependent manner. It activates glucocerebrosidase to decrease glucosylceramide accumulation in fibroblasts and lymphoblasts isolated from patients with Gaucher and Fabry disease, respectively.

C16 globotriaosylceramide is an endogenous sphingolipid found in mammalian cell membranes that is synthesized from C16 lactosylceramide . C16 globotriaosylceramide acts as a receptor for Shiga toxin in B cell-derived Raji cells and THP-1 monocytes. It accumulates in endothelial cells, pericytes, vascular smooth muscle cells, renal epithelial cells, dorsal ganglia neuronal cells, and myocardial cells in patients with Fabry disease. C16 globotriaosylceramide is also upregulated in plasma of patients with ovarian carcinoma compared to those with benign ovarian tumors or uterine fibroids.

C17 Globotriaosylceramide is a sphingolipid that has been used as an internal standard for the quantification of globotriaosylceramides in plasma and urine from patients with Fabry disease by GC-MS.

Globotriaosycleramides are glycosphingolipids found in mammalian cell membranes that are synthesized from lactosylceramides . They act as receptors for Shiga and Shiga-like toxins in vitro and in vivo. Globotriaosylceramides accumulate in endothelial cells, pericytes, vascular smooth muscle cells, renal epithelial cells, dorsal ganglia neuronal cells, and myocardial cells in patients with Fabry disease, a lysosomal storage disorder characterized by a deficiency in the enzyme α-galactosidase A. Globotriaosylceramides act as natural resistance factors to HIV infection, interacting with HIV gp120 to prevent its interaction with chemokine co-receptors and subsequent fusion of HIV to host cell membranes. This product contains a mixture of hydroxy and non-hydroxy fatty acid-containing globotriaosylceramides isolated from porcine red blood cells (RBCs).

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.

Ibiglustat (GZ402671) is a potent and selective Glucosylceramide synthase inhibitor and ceramide glucosyltransferase inhibitor. Ibiglustat blocks the formation of glucosylceramide (GL-1), a key intermediate in the synthesis of GL-3. Ibiglustat is potentially useful for treating Fabry disease. Fabry disease is a rare lysosomal storage disorder, which results in abnormal tissue deposits of a particular fatty substance called globotriaosylceramide (GL-3 or Gb3) throughout the body.

4-Methylumbelliferyl-α-D-Galactopyranoside (4MU-α-Gal) (4-MU-α-Gal) is a fluorogenic substrate of α-galactosidase. In addition to its use in characterizing novel α-galactosidases, 4-MU-α-Gal is used in assays to evaluate deficiency in α-galactosidase activity related to Fabry disease.

Ibiglustat (Venglustat) succinate is an orally active, brain-penetrant inhibitor of glucosylceramide synthase (GCS). It is utilized in the investigation of Gaucher disease type 3, Parkinson's disease associated with GBA mutations, Fabry disease, GM2 gangliosidosis, and autosomal dominant polycystic kidney disease.

Migalastat hydrochloride (GR181413A) is an orally available, potent and competitive inhibitor of alpha-galactosidase A. It promotes the transport of alpha-galactosidase A to the lysosome and can be used in the study of Fabry disease.

Lucerastat is a very effective and selective α-gal A (α-D-galactosidase) inhibitor for the treatment of lipid storage diseases and Fabry disease.

Globotriaosycleramides are glycosphingolipids found in mammalian cell membranes that are synthesized from lactosylceramides . They act as receptors for Shiga and Shiga-like toxins in vitro and in vivo. Globotriaosylceramides accumulate in endothelial cells, pericytes, vascular smooth muscle cells, renal epithelial cells, dorsal ganglia neuronal cells, and myocardial cells in patients with Fabry disease, a lysosomal storage disorder characterized by a deficiency in the enzyme α-galactosidase A. Globotriaosylceramides act as natural resistance factors to HIV infection, interacting with HIV gp120 to prevent its interaction with chemokine co-receptors and subsequent fusion of HIV to host cell membranes. This product contains a mixture of hydroxy fatty acid-containing globotriaosylceramides isolated from porcine red blood cells (RBCs).

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.

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.

L-threo Lysosphingomyelin (d18:1) (L-threo-Sphingosylphosphorylcholine) is a naturally occurring bioactive sphingolipid. It acts 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)