Isofagomine in vivo effects in a neuronopathic Gaucher disease mouse.

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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Bibliographic Details
Main Authors: Ying Sun, Huimin Ran, Benjamin Liou, Brian Quinn, Matt Zamzow, Wujuan Zhang, Jacek Bielawski, Kazuyuki Kitatani, Kenneth D R Setchell, Yusuf A Hannun, Gregory A Grabowski
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2011-04-01
Series:PLoS ONE
Online Access:https://www.ncbi.nlm.nih.gov/pmc/articles/pmid/21533102/pdf/?tool=EBI

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https://www.ncbi.nlm.nih.gov/pmc/articles/pmid/21533102/pdf/?tool=EBI

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