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A Topological Switch Enables Misfolding of the Cystic Fibrosis Transmembrane Conductance Regulator
[article]
2020
bioRxiv
pre-print
Cystic Fibrosis (CF) is a common lethal genetic disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel. Misfolding and degradation of CFTR are the hallmarks of the predominant mutation, F508del, located in the first nucleotide binding domain (NBD1). While the mutation is known to affect the thermal stability of NBD1 and assembly of CFTR domains, the molecular events that lead to misfolding of F508del-CFTR remain elusive. Here, we demonstrate
doi:10.1101/2020.07.09.195099
fatcat:njlar4o4vjexvnfpq4cplsiuuq