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The gating of the CFTR channel

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Abstract

Cystic fibrosis transmembrane conductance regulator (CFTR) is an anion channel expressed in the apical membrane of epithelia. Mutations in the CFTR gene are the cause of cystsic fibrosis. CFTR is the only ABC-protein that constitutes an ion channel pore forming subunit. CFTR gating is regulated in complex manner as phosphorylation is mandatory for channel activity and gating is directly regulated by binding of ATP to specific intracellular sites on the CFTR protein. This review covers our current understanding on the gating mechanism in CFTR and illustrates the relevance of alteration of these mechanisms in the onset of cystic fibrosis.

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Acknowledgments

Thanks to prof. Paolo Tammaro for critically reading the manuscript. This work was partially supported by the Italian Cystic Fibrosis Foundation (FCC#4/2014).

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  1. Istituto di Biofisica, CNR. Via De Marini, 6, 16149, Genoa, Italy

    Oscar Moran

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Moran, O. The gating of the CFTR channel. Cell. Mol. Life Sci. 74, 85–92 (2017). https://doi.org/10.1007/s00018-016-2390-z

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