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Migalastat-d5

CAT: 0804-HY-W728096Size: 1 EachDry Ice: NoHazardous: No
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CAT#:0804-HY-W728096Size:1 Each
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24/48H Stock Items & 2 to 6 Weeks non Stock Items.
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Description
Migalastat-d5 (GR181413A-d5 (free base) ) is deuterium labeled Migalastat. Migalastat (GR181413A free base) is an orally active α-galactosidase A molecular chaperone, with an IC50 value of 0.04 μM for human α-Gal A. Migalastat binds to the active site of certain unstable mutant forms of α-galactosidase A, facilitating their transport to the lysosome. After dissociation in the acidic environment, Migalastat enables the mutant α-galactosidase A to exhibit biological activity[1].
Product Name Alternative
GR181413A-d5 (free base)
UNSPSC
12352005
Target
Glycosidase; Isotope-Labeled Compounds
Related Pathways
Metabolic Enzyme/Protease; Others
Applications
Metabolism-protein/nucleotide metabolism
Field of Research
Others
Smiles
O[C@]1(CN[C@@H]([C@](O)([C@]1([2H])O)[2H])C(O)([2H])[2H])[2H]
Molecular Formula
C6H8D5NO4
Molecular Weight
168.20
References & Citations
[1]Russak EM, et al. Impact of Deuterium Substitution on the Pharmacokinetics of Pharmaceuticals. Ann Pharmacother. 2019;53 (2) :211-216.|[2]Asano N, et al. In vitro inhibition and intracellular enhancement of lysosomal alpha-galactosidase A activity in Fabry lymphoblasts by 1-deoxygalactonojirimycin and its derivatives. Eur J Biochem. 2000 Jul;267 (13) :4179-86.|[3]Ishii S, et al. Preclinical efficacy and safety of 1-deoxygalactonojirimycin in mice for Fabry disease. J Pharmacol Exp Ther. 2009 Mar;328 (3) :723-31.|[4]Young-Gqamana B, et al. Migalastat HCl reduces globotriaosylsphingosine (lyso-Gb3) in Fabry transgenic mice and in the plasma of Fabry patients. PLoS One. 2013;8 (3) :e57631.|[5]Welford RWD, et al. Glucosylceramide synthase inhibition with lucerastat lowers globotriaosylceramide and lysosome staining in cultured fibroblasts from Fabry patients with different mutation types. Hum Mol Genet. 2018 Oct. 27 (19) :3392-3403.
Shipping Conditions
Room temperature
Scientific Category
Isotope-Labeled Compounds
Clinical Information
No Development Reported

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