脱氧半乳糖野生霉素 盐酸盐
| 中文名称 | 脱氧半乳糖野生霉素 盐酸盐 |
|---|---|
| 中文同义词 | 1-脱氧半乳糖野尻霉素盐酸盐;脱氧半乳糖野生霉素 盐酸盐;1-DEOXYGALACTONOJIRIMYCIN(盐酸盐);化合物 T12038;脱氧半乳糖诺吉霉素;1-脱氧半乳糖诺吉霉素盐酸盐;(2R,3S,4R,5S)-2-(羟甲基)哌啶-3,4,5-三醇盐酸盐;(2R,3S,4R,5S)-2-甲醇哌啶-3,4,5-三醇盐酸盐 |
| 英文名称 | Deoxygalactonojirimycin Hydrochloride |
| 英文同义词 | 1,5-DIDEOXY-1,5-IMINO-D-GALACTITOL, HYDROCHLORIDE;1-DEOXYGALACTONOJIRIMYCIN HCL;DEOXYGALACTONOJIRIMYCIN HCL;DEOXYGALACTONOJIRIMYCIN, HYDROCHLORIDE;DGJ;DGJ, HYDROCHLORIDE;GALACTOSTATIN HCL;3,4,5-Piperidinetriol, 2-(hydroxymethyl)-, hydrochloride, (2R,3S,4R,5S)- |
| CAS号 | 75172-81-5 |
| 分子式 | C6H13NO4.ClH |
| 分子量 | 199.63 |
| EINECS号 | |
| 相关类别 | 生化试剂;13C & 2H Sugars;Carbohydrates & Derivatives;Glycosidase Inhibitors;Inhibitors |
| Mol文件 | 75172-81-5.mol |
| 结构式 |  |
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.
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英文
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| 更新日期 | 产品编号 | 产品名称 | CAS号 | 包装 | 价格 |
|---|---|---|---|---|---|
| 2026/06/05 | HY-14929A | 脱氧半乳糖野生霉素 盐酸盐 Migalastat hydrochloride | 75172-81-5 | 1 mg | 386元 |
| 2026/06/05 | HY-14929A | 脱氧半乳糖野生霉素 盐酸盐 Migalastat hydrochloride | 75172-81-5 | 5mg | 750元 |