Isofagomine D-Tartrate

Isofagomine tartrate (957230-65-8) is a competitive inhibitor of human lysosomal β-glucosidase, IC50= 0.06 μM.1Increases the activity of N370S mutant acid β-glucosidase (GlcCerase) in Gaucher fibroblasts.2Displays a direct pharmacological chaperone effect greatly increasing the amount of newly synthesized GlcCerase that traffics out of the ER.2

Isofagomine D-Tartrate is Off-White Solid

Isofagomine D-Tartrate is used as human acid glucosidase inhibitor and for the treatment of Gaucher disease

Used as human acid β-glucosidase inhibitor and for the treatment of Gaucher disease.

[1] CHINAMI KURIYAMA . In vitro inhibition of glycogen-degrading enzymes and glycosidases by six-membered sugar mimics and their evaluation in cell cultures[J]. Bioorganic & Medicinal Chemistry, 2008, 16 15: Pages 7330-7336. DOI:10.1016/j.bmc.2008.06.026
[2] RICHARD A STEET. The iminosugar isofagomine increases the activity of N370S mutant acid beta-glucosidase in Gaucher fibroblasts by several mechanisms.[J]. Proceedings of the National Academy of Sciences of the United States of America, 2006, 103 37: 13813-13818. DOI:10.1073/pnas.0605928103