Article
A novel model for the first nucleotide binding domain of the cystic fibrosis transmembrane conductance regulator.
FEBS letters - 5 May 1997
Annereau J P, Wulbrand U, Vankeerberghen A, Cuppens H, Bontems F, Tümmler B, Cassiman J J, Stoven V
Abstract excerpt
Cystic fibrosis is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The most frequent mutation is the deletion of F508 in the first nucleotide binding fold (NBF1). It induces a perturbation in the folding of NBF1, which impedes posttranslational maturation of CFTR. Determination of the three-dimensional structure of NBF1 would help to understand this defect. We present a...
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