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VX-809 corrects folding defects in cystic fibrosis transmembrane conductance regulator protein through action on membrane-spanning domain 1
Cystic fibrosis (CF) is a fatal genetic disorder associated with defective hydration of lung airways due to the loss of chloride transport through the CF transmembrane conductance regulator protein (CFTR). CFTR contains two membrane-spanning domains (MSDs), two nucleotide-binding domains (NBDs), and...
Autores principales: | Ren, Hong Yu, Grove, Diane E., De La Rosa, Oxana, Houck, Scott A., Sopha, Pattarawut, Van Goor, Fredrick, Hoffman, Beth J., Cyr, Douglas M. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
The American Society for Cell Biology
2013
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3784376/ https://www.ncbi.nlm.nih.gov/pubmed/23924900 http://dx.doi.org/10.1091/mbc.E13-05-0240 |
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