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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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| Main Authors: | , , , , , |
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| Formato: | Artigo |
| Idioma: | Inglês |
| Publicado em: |
2006
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| Assuntos: | |
| Acesso em linha: | 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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