期刊论文详细信息
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