Lyso-Globotriaosylceramide (d18:1)

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 (PMA, Cay-10008014) 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 alpha-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 alpha-galactosidase inhibitor 1-deoxygalactonojirimycin (migalastat, Cay-17179) 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. [Matreya, LLC. Catalog No. 1520]Formal Name: (2S,3R,4E)-2-amino-3-hydroxy-4-octadecen-1-yl O-alpha-D-galactopyranosyl-(1->4)-O-beta-D-galactopyranosyl-(1->4)-beta-D-glucopyranoside. CAS Number: 126550-86-5. Synonyms: Globotriaosylsphingosine (d18:1), Lyso-Ceramide Trihexoside, Lyso-Gb3. Molecular Formula: C36H67NO17. Formula Weight: 785.9. Purity: >98%. Formulation: (Request formulation change), A solid. Solubility: Chloroform:Methanol:Water (4:3:1): Soluble. SMILES: O[C@H]1[C@H](O[C@]2([H])O[C@H](CO)[C@H](O[C@]3([H])[C@H](O)[C@@H](O)[C@@H](O)[C@@H](CO)O3)[C@H](O)[C@H]2O)[C@@H](CO)O[C@@H](OC[C@H](N)[C@H](O)/C=C/CCCCCCCCCCCCC)[C@@H]1O. InChi Code: InChI=1S/C36H67NO17/c1-2-3-4-5-6-7-8-9-10-11-12-13-14-15-21(41)20(37)19-49-34-30(47)27(44)32(23(17-39)51-34)54-36-31(48)28(45)33(24(18-40)52-36)53-35-29(46)26(43)25(42)22(16-38)50-35/h14-15,20-36,38-48H,2-13,16-19,37H2,1H3/b15-14+/t20-,21+,22+,23+,24+,25-,26-,27+,28+,29+,30+,31+,32+,33-,34+,35+,36-/m0/s1. InChi Key: GRGNVOCPFLXGDQ-TWHXEDJUSA-N.