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Isofagomine In Vivo Effects in a Neuronopathic Gaucher Disease Mouse
The pharmacological chaperone, isofagomine (IFG), enhances acid β-glucosidase (GCase) function by altering folding, trafficking, and activity in wild-type and Gaucher disease fibroblasts. The in vivo effects of IFG on GCase activity, its substrate levels, and phenotype were evaluated using a neurono...
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Autori principali: | , , , , , , , , , , |
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Natura: | Artigo |
Lingua: | Inglês |
Pubblicazione: |
Public Library of Science
2011
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Soggetti: | |
Accesso online: | https://ncbi.nlm.nih.gov/pmc/articles/PMC3080394/ https://ncbi.nlm.nih.gov/pubmed/21533102 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0019037 |
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