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 function of the protein. However, substitution of the Phe508 with small uncharged amino acids, including cysteine, is...
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