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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Detalles Bibliográficos
Autores principales: Sun, Ying, Ran, Huimin, Liou, Benjamin, Quinn, Brian, Zamzow, Matt, Zhang, Wujuan, Bielawski, Jacek, Kitatani, Kazuyuki, Setchell, Kenneth D. R., Hannun, Yusuf A., Grabowski, Gregory A.
Formato: Texto
Lenguaje:English
Publicado: Public Library of Science 2011
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3080394/
https://www.ncbi.nlm.nih.gov/pubmed/21533102
http://dx.doi.org/10.1371/journal.pone.0019037

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3080394/
https://www.ncbi.nlm.nih.gov/pubmed/21533102
http://dx.doi.org/10.1371/journal.pone.0019037

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