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Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Potentiators Protect G551D but Not ΔF508 CFTR from Thermal Instability
[Image: see text] The G551D cystic fibrosis transmembrane conductance regulator (CFTR) mutation is associated with severe disease in ∼5% of cystic fibrosis patients worldwide. This amino acid substitution in NBD1 results in a CFTR chloride channel characterized by a severe gating defect that can be...
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| Main Authors: | , |
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| Formato: | Artigo |
| Idioma: | Inglês |
| Publicado em: |
American
Chemical Society
2014
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| Acesso em linha: | https://ncbi.nlm.nih.gov/pmc/articles/PMC4159205/ https://ncbi.nlm.nih.gov/pubmed/25148434 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1021/bi501007v |
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