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Rescue of Murine F508del CFTR Activity in Native Intestine by Low Temperature and Proteasome Inhibitors
Most patients with Cystic Fibrosis (CF) carry at least one allele with the F508del mutation, resulting in a CFTR chloride channel protein with a processing, gating and stability defect, but with substantial residual activity when correctly sorted to the apical membranes of epithelial cells. New ther...
Autores principales: | Wilke, Martina, Bot, Alice, Jorna, Huub, Scholte, Bob J., de Jonge, Hugo R. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2012
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3528711/ https://www.ncbi.nlm.nih.gov/pubmed/23284872 http://dx.doi.org/10.1371/journal.pone.0052070 |
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