Article
Rattlesnake Phospholipase A2 Increases CFTR-Chloride Channel Current and Corrects ∆F508CFTR Dysfunction: Impact in Cystic Fibrosis.
Journal of molecular biology - 17 Jul 2016
Faure Grazyna, Bakouh Naziha, Lourdel Stéphane, Odolczyk Norbert, Premchandar Aiswarya, Servel Nathalie, Hatton Aurélie, Ostrowski Maciej K, Xu Haijin, Saul Frederick A, Moquereau Christelle, Bitam Sara, Pranke Iwona, Planelles Gabrielle, Teulon Jacques, Herrmann Harald, Roldan Ariel, Zielenkiewicz Piotr, Dadlez Michal, Lukacs Gergely L, Sermet-Gaudelus Isabelle, Ollero Mario, Corringer Pierre-Jean, Edelman Aleksander
Abstract excerpt
Deletion of Phe508 in the nucleotide binding domain (∆F508-NBD1) of the cystic fibrosis transmembrane regulator (CFTR; a cyclic AMP-regulated chloride channel) is the most frequent mutation associated with cystic fibrosis. This mutation affects the maturation and gating of CFTR protein. The search for new high-affinity ligands of CFTR acting as dual modulators (correctors/activators) presents a major challenge in...
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