产品
编 号：F029762
分子式：C6H13NO4
分子量：163.17
分子式：C6H13NO4
分子量：163.17
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CAS No: 108147-54-2
产品详情
生物活性：
Migalastat (GR181413A free base) is an orally active and competitive inhibitor of α-galactosidase A (α-Gal A) with an IC50 of 0.04 μM for human α-Gal A.
体内研究：
Fabry disease is an X-linked recessive disorder caused by the deficient activity of α-galactosidase A.Migalastat (oral gavage, 3 mg/kg daily for 4 weeks) increases α-Gal A activity in heart, kidney, spleen, and liver in a dose- and time-dependently in transgenic mice that express human mutant alpha-Gal A (TgM).Migalastat shows the half-life of less than 1 day in all major issues in TgM for 2 weeks pretreatment.Migalastat (oral gavage, 100 mg/kg daily for 28 days) to transgenic mice reduces lyso-Gb3 levels up to 64%, 59%, and 81% in kidney, heart, and skin, respectively.Animal Model:Male nontransgenic (Non-Tg) C57BL/6 mice; transgenic mice expressing human mutant R301Q α-Gal A (TgM), α-Gal A knockout mice (KO), mice express human R301Q α-Gal A in a null background (TgM/KO)
Dosage:3 mg/kg
Administration:Oral gavage; every day for 4 weeks
Result:Reduced Globotriaosylceramide (Gb3) storage remarkably in kidney of mice.
体外研究：
Migalastat inhibits human lysosomal a-Gal A with IC50 and Ki values of 0.04 μM.
Migalastat (GR181413A free base) is an orally active and competitive inhibitor of α-galactosidase A (α-Gal A) with an IC50 of 0.04 μM for human α-Gal A.
体内研究：
Fabry disease is an X-linked recessive disorder caused by the deficient activity of α-galactosidase A.Migalastat (oral gavage, 3 mg/kg daily for 4 weeks) increases α-Gal A activity in heart, kidney, spleen, and liver in a dose- and time-dependently in transgenic mice that express human mutant alpha-Gal A (TgM).Migalastat shows the half-life of less than 1 day in all major issues in TgM for 2 weeks pretreatment.Migalastat (oral gavage, 100 mg/kg daily for 28 days) to transgenic mice reduces lyso-Gb3 levels up to 64%, 59%, and 81% in kidney, heart, and skin, respectively.Animal Model:Male nontransgenic (Non-Tg) C57BL/6 mice; transgenic mice expressing human mutant R301Q α-Gal A (TgM), α-Gal A knockout mice (KO), mice express human R301Q α-Gal A in a null background (TgM/KO)
Dosage:3 mg/kg
Administration:Oral gavage; every day for 4 weeks
Result:Reduced Globotriaosylceramide (Gb3) storage remarkably in kidney of mice.
体外研究：
Migalastat inhibits human lysosomal a-Gal A with IC50 and Ki values of 0.04 μM.
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