脱氧半乳糖野生霉素 盐酸盐
脱氧半乳糖野生霉素 盐酸盐 性质
| 熔点 | 260 |
|---|---|
| 储存条件 | 2-8°C |
| 溶解度 | 甲醇（微溶）、水（微溶） |
| 形态 | 白色结晶固体 |
| 颜色 | 白色至棕色 |
| 生物来源 | synthetic (organic) |
| 水溶解性 | water: soluble 1mg/mL |
| InChI | 1S/C6H13NO4.ClH/c8-2-3-5(10)6(11)4(9)1-7-3;/h3-11H,1-2H2;1H/t3-,4+,5+,6-;/m1./s1 |
| InChIKey | ZJIHMALTJRDNQI-OLALXQGDSA-N |
| SMILES | Cl.OC[C@H]1NC[C@H](O)[C@@H](O)[C@H]1O |
脱氧半乳糖野生霉素 盐酸盐 用途与合成方法
脱氧半乳糖野生霉素盐酸盐是一种有效和竞争性的α-galactosidase A抑制剂，对人α-Gal A的 IC50值为0.04 μM。
IC50: 0.04 μM (human α-Gal A); Ki: 0.04 μM (human α-Gal A)
Both IC 50 and K i values of Migalastat (GR181413A) hydrochloride toward human lysosomal a-Gal A are 0.04 μM.
Fabry disease is an X-linked recessive disorder caused by the deficient activity of α-galactosidase A. α-Gal A activity in heart, kidney, spleen, and liver is increased dose- and time-dependently in transgenic mice that express human mutant alpha-Gal A with Migalastat (GR181413A) hydrochloride treatment. The half-life of DGJ is less than 1 day in all major issues and that of the enzyme synthesized during the DGJ treatment period is approximately 4 days. Oral administration of Migalastat (GR181413A) hydrochloride reduces tissue GL-3 in fabry transgenic mice, and in urine and kidneys of some FD patients. Oral administration of Migalastat (GR181413A) hydrochloride to transgenic mice reduces elevated lyso-Gb3 levels up to 64%, 59%, and 81% in kidney, heart, and skin, respectively, generally equal to or greater than observed for GL-3.
脱氧半乳糖野生霉素 盐酸盐 价格(试剂级)
| 更新日期 | 产品编号 | 产品名称 | CAS号 | 包装 | 价格 |
|---|---|---|---|---|---|
| 2025-12-22 | HY-14929A | 脱氧半乳糖野生霉素 盐酸盐 | 75172-81-5 | 1 mg | 386 |
| 2025-12-22 | HY-14929A | 脱氧半乳糖野生霉素 盐酸盐 | 75172-81-5 | 5mg | 750 |
