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Compounds that correct F508del-CFTR trafficking can also correct other protein trafficking diseases: an in vitro study using cell lines
BACKGROUND: Many genetic diseases are due to defects in protein trafficking where the mutant protein is recognized by the quality control systems, retained in the endoplasmic reticulum (ER), and degraded by the proteasome. In many cases, the mutant protein retains function if it can be trafficked to...
Autores principales: | Sampson, Heidi M, Lam, Hung, Chen, Pei-Chun, Zhang, Donglei, Mottillo, Cristina, Mirza, Myriam, Qasim, Karim, Shrier, Alvin, Shyng, Show-Ling, Hanrahan, John W, Thomas, David Y |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2013
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3558398/ https://www.ncbi.nlm.nih.gov/pubmed/23316740 http://dx.doi.org/10.1186/1750-1172-8-11 |
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