Molecular structure of the ATP-bound, phosphorylated human CFTR

Mutations in cystic fibrosis transmembrane conductance regulator (CFTR) cause cystic fibrosis, a lethal genetic disease occurring in people of northern European descent. Decades of study have been directed toward a molecular understanding of this ion channel. The structure presented here enables a direct correlation of structure with function, most of which has been characterized in human CFTR.