Malfunction of cystic fibrosis transmembrane conductance regulator (CFTR), a member of the ABC protein superfamily that functions as an ATP-gated chloride channel, causes the lethal genetic disease, cystic fibrosis. This review focuses on the most recent findings on the gating mechanism of CFTR. Potential clinical relevance and implications to ABC transporter function are also discussed.Ion channels and active transporters are integral membrane proteins ubiquitously expressed in every living cell. They allow trafficking and communications between the intracellular and extracellular environments that would be otherwise isolated by the plasma membrane. Traditionally, channels and transporters are thought to be completely different entities. For ion channels, it can be as simple as a tunnel-like structure embedded in the lipid bilayer with the addition of a regulatable gate to control thepatency of the tunnel.
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