Isofagomine In Vivo Effects in a Neuronopathic Gaucher Disease Mouse
Isofagomine In Vivo Effects in a Neuronopathic Gaucher Disease Mouse
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United States: Public Library of Science
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Language
English
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United States: Public Library of Science
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Contents
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 neuronopathic Gaucher disease mouse model, 4L;C* (V394L/V...
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Full title
Isofagomine In Vivo Effects in a Neuronopathic Gaucher Disease Mouse
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TN_cdi_plos_journals_1297617108
Permalink
https://devfeature-collection.sl.nsw.gov.au/record/TN_cdi_plos_journals_1297617108
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ISSN
1932-6203
E-ISSN
1932-6203
DOI
10.1371/journal.pone.0019037
