| CAS NO: | 126550-86-5 |
| 包装: | 1mg |
| 市场价: | 9813元 |
| Cas No. | 126550-86-5 |
| Canonical 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 |
| 分子式 | C36H67NO17 |
| 分子量 | 785.9 |
| 溶解度 | Chloroform:Methanol:Water (4:3:1): Soluble |
| 储存条件 | Store at -20°C |
| General tips | For obtaining a higher solubility , please warm the tube at 37 ℃ and shake it in the ultrasonic bath for a while. |
| Shipping Condition | Evaluation sample solution : ship with blue ice All other available size: ship with RT , or blue ice upon request |
| 产品描述 | 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. |
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