Remodeling the Proteostasis Network to Rescue Glucocerebrosidase Variants by Inhibiting ER-Associated Degradation and Enhancing ER Folding

Gaucher’s disease (GD) is characterized by loss of lysosomal glucocerebrosidase (GC) activity. Mutations in the gene encoding GC destabilize the protein’s native folding leading to ER-associated degradation (ERAD) of the misfolded enzyme. Enhancing the cellular folding capacity by remodeling the pro...

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Detalles Bibliográficos
Autores principales: Wang, Fan, Segatori, Laura
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2013
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3631227/
https://www.ncbi.nlm.nih.gov/pubmed/23620750
http://dx.doi.org/10.1371/journal.pone.0061418

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3631227/
https://www.ncbi.nlm.nih.gov/pubmed/23620750
http://dx.doi.org/10.1371/journal.pone.0061418

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