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lüll The ABC protein turned chloride channel whose failure causes cystic fibrosis Gadsby DC; Vergani P; Csanady LNature 2006[Mar]; 440 (7083): 477-83CFTR chloride channels are encoded by the gene mutated in patients with cystic fibrosis. These channels belong to the superfamily of ABC transporter ATPases. ATP-driven conformational changes, which in other ABC proteins fuel uphill substrate transport across cellular membranes, in CFTR open and close a gate to allow transmembrane flow of anions down their electrochemical gradient. New structural and biochemical information from prokaryotic ABC proteins and functional information from CFTR channels has led to a unifying mechanism explaining those ATP-driven conformational changes.|ATP-Binding Cassette Transporters/chemistry/genetics/*metabolism[MESH]|Adenosine Triphosphate/metabolism[MESH]|Animals[MESH]|Binding Sites[MESH]|Cystic Fibrosis Transmembrane Conductance Regulator/chemistry/genetics/*metabolism[MESH]|Cystic Fibrosis/etiology/genetics/*metabolism[MESH]|Humans[MESH]|Hydrolysis[MESH]|Ion Channel Gating[MESH]|Mutation[MESH]|Nucleotides/metabolism[MESH]|Phosphorylation[MESH]|Protein Conformation[MESH]|Protein Structure, Tertiary[MESH] |