Article
Nucleotide binding domains of human CFTR: a structural classification of critical residues and disease-causing mutations.
Cellular and molecular life sciences : CMLS - 1 Sept 2005
Eudes R, Lehn P, Férec C, Mornon J-P, Callebaut I
Abstract excerpt
Defective function of the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) causes CF, the most frequent lethal inherited disease among the Caucasian population. The structure of this chloride ion channel includes two nucleotide-binding domains (NBDs), whose ATPase activity controls channel gating. Recently, the experimental structures of mouse and human CFTR NBD1 and our model of the human CFTR...
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