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A truncated CFTR protein rescues endogenous ΔF508-CFTR and corrects chloride transport in mice

Cystic fibrosis (CF) is most frequently associated with deletion of phenylalanine at position 508 (ΔF508) in the CF transmembrane conductance regulator (CFTR) protein. The ΔF508-CFTR mutant protein exhibits a folding defect that affects its processing and impairs chloride-channel function. This stud...

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Autori principali: Cormet-Boyaka, Estelle, Hong, Jeong S., Berdiev, Bakhram K., Fortenberry, James A., Rennolds, Jessica, Clancy, J. P., Benos, Dale J., Boyaka, Prosper N., Sorscher, Eric J.
Natura: Artigo
Lingua:Inglês
Pubblicazione: The Federation of American Societies for Experimental Biology 2009
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Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC2775001/
https://ncbi.nlm.nih.gov/pubmed/19620404
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1096/fj.08-127878
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