| Bioactivity | Globotriaosylsphingosine (lyso-Gb3), triacylsphingosine globulin, is a glycosphingolipid biomarker. Alpha-galactosidase A deficiency causes Fabry disease, an X-linked lysosomal storage disorder. A characteristic feature of Fabry disease is the abnormal accumulation of Globotriaosylsphingosine in the urine of the organism. Globotriaosylsphingosine also inhibits KCa3.1 channel expression, resulting in reduced Ca2+ levels. Thus, Globotriaosylsphingosine inhibits the differentiation of myofibroblasts into myofibroblasts and inhibits collagen synthesis in fibroblasts. Globotriaosylsphingosine is useful in the study of ascending thoracic aortic aneurysms in Fabry disease[1][2]. |
| Target | KCa3.1 |
| Invitro | Globotriaosylsphingosine (100 nM, 500 nM; 24 h) 降低 KCa3.1 mRNA 表达水平和 Glo1a1 和 Glo3a1 的蛋白水平[1]。Globotriaosylsphingosine (50-500 nM, 24 h) 剂量依赖性抑制成纤维细胞生长、分化为肌成纤维细胞和胶原蛋白合成[1]。 0 --> Globotriaosylsphingosine 相关抗体: |
| Name | Globotriaosylsphingosine |
| CAS | 126550-86-5 |
| Formula | C36H67NO17 |
| Molar Mass | 785.91 |
| Transport | Room temperature in continental US; may vary elsewhere. |
| Storage | Please store the product under the recommended conditions in the Certificate of Analysis. |
| Reference | [1]. Choi JY, et al. Lyso-globotriaosylceramide downregulates KCa3.1 channel expression to inhibit collagen synthesis in fibroblasts. Biochem Biophys Res Commun. 2015 Dec 25;468(4):883-8. [2]. Auray-Blais C, et al. How well does urinary lyso-Gb3 function as a biomarker in Fabry disease? Clin Chim Acta. 2010 Dec 14;411(23-24):1906-14. |
Globotriaosylsphingosine
CAS: 126550-86-5 F: C36H67NO17 W: 785.91
Globotriaosylsphingosine (lyso-Gb3), triacylsphingosine globulin, is a glycosphingolipid biomarker. Alpha-galactosidase
Sales Email：peptidedb@qq.com
This product is for research use only, not for human use. We do not sell to patients.