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Ex Vivo and in Vivo Effects of Isofagomine on Acid β-Glucosidase Variants and Substrate Levels in Gaucher Disease

Isofagomine (IFG) is an acid β-glucosidase (GCase) active site inhibitor that acts as a pharmacological chaperone. The effect of IFG on GCase function was investigated in GCase mutant fibroblasts and mouse models. IFG inhibits GCase with K(i) ∼30 nm for wild-type and mutant enzymes (N370S and V394L)...

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Autors principals: Sun, Ying, Liou, Benjamin, Xu, You-Hai, Quinn, Brian, Zhang, Wujuan, Hamler, Rick, Setchell, Kenneth D. R., Grabowski, Gregory A.
Format: Artigo
Idioma:Inglês
Publicat: American Society for Biochemistry and Molecular Biology 2012
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Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC3281716/
https://ncbi.nlm.nih.gov/pubmed/22167193
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M111.280016
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