南京辰瑞晟生物医药科技有限公司
司美格鲁肽 , 阿法诺肽 , 索玛鲁肽 , 他达那非 , 甾体激素原料
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

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)-
CAS75172-81-5
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.

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发布时间:2024-11-22
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