1-脱氧半乳糖野尻霉素盐酸盐CAS: 75172-81-5原料仅供出口或科研试验

发布：2023-08-29 14:31，更新：2024-05-18 07:00

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	Methanol (Slightly), Water (Slightly)
存储条件	2-8°C
外观	White crystalline solid
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.