Chaperone displacement from mutant cystic fibrosis transmembrane conductance regulator restores its function in human airway epithelia

Other literature type English OPEN
Sun, Fei; Mi, Zhibao; Condliffe, Steven B.; Bertrand, Carol A.; Gong, Xiaoyan; Lu, Xiaoli; Zhang, Ruilin; Latoche, Joseph D.; Pilewski, Joseph M.; Robbins, Paul D.; Frizzell, Raymond A.;
  • Publisher: The Federation of American Societies for Experimental Biology
  • Related identifiers: pmc: PMC2518260, doi: 10.1096/fj.07-105338
  • Subject: Research Communications
    mesheuropmc: respiratory system

Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause cystic fibrosis (CF). The most common mutation, ΔF508, omits the phenylalanine residue at position 508 in the first nucleotide binding domain (NBD1) of CFTR. The mutant protein is ret... View more
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