Figure 1.

Structure of CFTR in its ATP-free, unphosphorylated closed conformation and ATP-bound open conformation, in complex with the VX770 (ivacaftor) potentiator

Transmembrane domains (TMDs) and nucleotide-binding domains (NBDs) are represented by green and slate cartoons and labeled. The residues mutated in this study (W57 and A234) are represented by red sticks and labeled. VX770 and Mg²⁺-ATP are represented by magenta and yellow sticks, respectively (cations by blue spheres), and labeled. The membrane bilayer is represented by shaded thick gray lines. The structural models have been obtained as explained in the methods section, using the 5UAK (closed CFTR) and 6O1V (open CFTR) pdb files as inputs.