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Folding and rescue of a cystic fibrosis transmembrane conductance regulator tra...

Impairment of the cystic fibrosis transmembrane conductance regulator (CFTR) Cl channel causes cystic fibrosis, a fatal genetic disease. Here, to gain insight into CFTR structure and function, we exploited interspecies differences between CFTR homologues using human (h)-murine (m) CFTR chimeras containing murine nucleotide-binding domains (NBDs) or regulatory domain on an hCFTR backbone. Among 15 hmCFTR chimer...