The ABC protein turned chloride channel whose failure causes cystic fibrosis.
basic_science · Level V
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- Record sourced from PubMed, PMID 16554808.
- Also identified by PMC identifier PMC2229623.
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Abstract
CFTR 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.