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Restoration of domain folding and interdomain assembly by second-site suppressors of the ΔF508 mutation in CFTR

Deletion of PHE508 (ΔF508) from the first nucleotide-binding domain (NBD1) of CFTR, which causes most cystic fibrosis, disrupts the folding and assembly of the protein. Although the folding pathways and yield of isolated NBD1 are altered, its global structure is not, and details of the changes in th...

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Autori principali: He, Lihua, Aleksandrov, Luba A., Cui, Liying, Jensen, Timothy J., Nesbitt, Kenneth L., Riordan, John R.
Natura: Artigo
Lingua:Inglês
Pubblicazione: The Federation of American Societies for Experimental Biology 2010
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Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC2909275/
https://ncbi.nlm.nih.gov/pubmed/20233947
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1096/fj.09-141788
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