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Selective Action of the Iminosugar Isofagomine, a Pharmacological Chaperone For Mutant Forms of Acid-β-Glucosidase

Gaucher disease is a lysosomal glycolipid storage disorder characterized by defects in acid-β-glucosidase (GlcCerase), the enzyme responsible for the catabolism of glucosylceramide. We recently demonstrated that isofagomine (IFG), an iminosugar that binds to the active site of GlcCerase, enhances th...

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Detalles Bibliográficos
Main Authors:	Steet, Richard, Chung, Stephen, Lee, Wang-Sik, Pine, Corey W., Do, Hung, Kornfeld, Stuart
Formato:	Artigo
Idioma:	Inglês
Publicado:	2006
Assuntos:	Article
Acceso en liña:	https://ncbi.nlm.nih.gov/pmc/articles/PMC1892903/ https://ncbi.nlm.nih.gov/pubmed/17217920 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.bcp.2006.12.015
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Selective Action of the Iminosugar Isofagomine, a Pharmacological Chaperone For Mutant Forms of Acid-β-Glucosidase

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