中文名 | 1-Deoxygalactonojirimycin Hydrochloride |
英文名 | 1-Deoxygalactonojirimycin Hydrochloride |
别名 | 化合物 T12038 脱氧半乳糖苷酶抑素盐酸盐 脱氧半乳糖野生霉素 盐酸盐 1-脱氧半乳糖野尻霉素盐酸盐 1-DEOXYGALACTONOJIRIMYCIN(盐酸盐) |
英文别名 | DGJ GALACTOSTATIN HCL DGJ, HYDROCHLORIDE DEOXYGALACTONOJIRIMYCIN HCL 1-DEOXYGALACTONOJIRIMYCIN HCL DEOXYGALACTONOJIRIMYCIN, HYDROCHLORIDE 1-Deoxygalactonojirimycin Hydrochloride 1,5-DIDEOXY-1,5-IMINO-D-GALACTITOL, HYDROCHLORIDE 3,4,5-Piperidinetriol, 2-(hydroxymethyl)-, hydrochloride, (2R,3S,4R,5S)- |
CAS | 75172-81-5 |
化学式 | C6H14ClNO4 |
分子量 | 199.63 |
熔点 | 260 |
溶解度 | 甲醇 (微溶) 、水 (微溶) |
存储条件 | 2-8°C |
外观 | 白色结晶固体 |
颜色 | White to Brown |
体外研究 | Both IC 50 and K i values of Migalastat (GR181413A) hydrochloride toward human lysosomal a-Gal A are 0.04 μM. |
体内研究 | 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. |
危险品标志 | Xi - 刺激性物品 |
风险术语 | 36/37/38 - 刺激眼睛、呼吸系统和皮肤。 |
安全术语 | S26 - 不慎与眼睛接触后,请立即用大量清水冲洗并征求医生意见。 S36 - 穿戴适当的防护服。 |
WGK Germany | 3 |
海关编号 | 29333990 |
Hazard Note | Irritant |
1mg | 5mg | 10mg | |
---|---|---|---|
1 mM | 5.009 ml | 25.046 ml | 50.093 ml |
5 mM | 1.002 ml | 5.009 ml | 10.019 ml |
10 mM | 0.501 ml | 2.505 ml | 5.009 ml |
5 mM | 0.1 ml | 0.501 ml | 1.002 ml |
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