脱氧半乳糖野生霉素 盐酸盐
- CAS号:75172-81-5
- 英文名:Deoxygalactonojirimycin Hydrochloride
- 中文名:脱氧半乳糖野生霉素 盐酸盐
- CBNumber:CB5252941
- 分子式:C6H13NO4.ClH
- 分子量:199.63
- MOL File:75172-81-5.mol
- 熔点 :260
- 储存条件 :2-8°C
- 溶解度 :Methanol (Slightly), Water (Slightly)
- 形态 :White crystalline solid
- 颜色 :White to Brown
脱氧半乳糖野生霉素 盐酸盐性质、用途与生产工艺
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用途
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。
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靶点
IC50: 0.04 μM (human α-Gal A); Ki: 0.04 μM (human α-Gal A)
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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.
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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.
- 更新日期:2024/11/08
- 产品编号:HY-14929A
- 产品名称:Migalastat hydrochloride
- CAS编号:
- 包装:1 mg
- 价格:386元
- 更新日期:2024/11/08
- 产品编号:HY-14929A
- 产品名称:脱氧半乳糖野生霉素 盐酸盐 Migalastat hydrochloride
- CAS编号:75172-81-5
- 包装:5mg
- 价格:750元
- 公司名称:GlycoFineChem
- 联系电话:--
- 电子邮件:glycofinechem@viclink.co.nz
- 国家:新西兰
- 产品数:43
- 优势度:30