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Migalastat hydrochloride

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Migalastat hydrochloride
结构式
75172-81-5
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中国地区超过5000个高品质化合物库存
应用领域
Migalastat hydrochloride (GR181413A) 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。
化学性质生物活性化合物的使用COA & MSDS相关产品产品说明书
Cas No.: 75172-81-5
名称:
别名: AT1001; AT 1001; AT-1001; GR181413A; GR 181413A; GR-181413A; 1,5-Dideoxy-1,5-imino-D-galactitol; 1-Deoxygalactonojirimycin; 1-Deoxygalactostatin; Amigal; Migalastat
SMILES: O[C@H]1[C@@H](CO)NC[C@H](O)[C@H]1O.Cl
分子式: C6H14ClNO4
分子量: 199.63
纯度:
保存条件: 2 years -20°C Powder, 2 weeks 4°C in DMSO, 6 months -80°C in DMSO
Description:
In Vivo:
In Vitro:
References: Both IC50 and Ki values of migalastat 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 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 hydrochloride reduces tissue GL-3 in fabry transgenic mice, and in urine and kidneys of some FD patients. Oral administration of migalastat 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.
Kinase Assay:
Cell Assay:
Animal Administration:
References:
MSDS
COA
LOT NO. DOWNLOAD
2018-0101
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