Article
The role of cystic fibrosis transmembrane conductance regulator phenylalanine 508 side chain in ion channel gating.
The Journal of physiology - 15 Apr 2006
Cui Liying, Aleksandrov Luba, Hou Yue-Xian, Gentzsch Martina, Chen Jey-Hsin, Riordan John R, Aleksandrov Andrei A
Abstract excerpt
Cystic fibrosis transmembrane conductance regulator (CFTR) is an ion channel employing the ABC transporter structural motif. Deletion of a single residue (Phe508) in the first nucleotide-binding domain (NBD1), which occurs in most patients with cystic fibrosis, impairs both maturation and functio...
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