The ABC protein turned chloride channel whose failure causes cystic fibrosis

Review

关键词: TRANSMEMBRANE CONDUCTANCE REGULATOR;    NUCLEOTIDE-BINDING DOMAINS;    CFTR CL-CHANNEL;    R-DOMAIN;    ATP-BINDING;    PHOSPHORYLATION SITES;    P-GLYCOPROTEIN;    CASSETTE TRANSPORTERS;    KINASE-A;    HYDROLYSIS;   

DOI  :  10.1038/nature04712

来源: SCIE

【 摘 要 】

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.

【 授权许可】

Free