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CFTR structure: lassoing cystic fibrosis

Nature Structural & Molecular Biology volume 24, pages 13–14 (2017)Cite this article

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Loss of function of the CFTR anion channel leads to cystic fibrosis, the most common inherited condition in humans of European origin. A recently reported structure for CFTR at 3.7-Å resolution reveals an unexpected 'lasso' domain and provides new insights into channel function in healthy individuals and in people with cystic fibrosis.

Proper regulation of the fluid layer adjacent to external surfaces of organs in the body is vital: too much fluid, and there is severe water loss (leading, for example, to diarrhea); too little, and there is blockage (as experienced during constipation). We have all suffered mild forms of these problems during our lives. People with cystic fibrosis, however, battle fluid dysregulation every day—in this case not in the intestine, but in the lungs. Here, not enough fluid means that cilia on the surface of the airways cannot beat to clear mucus from the lungs. The mucus becomes sticky and viscous. Because the normal role of mucus clearance is to eliminate potentially harmful bacteria and other pathogens, abnormal, stagnated mucus will lead to opportunistic, sometimes life-threatening, lung infections. The scarring caused by many such infections further reduces the capacity of the lungs for gas exchange.

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Figure 1: Zebrafish CFTR structure determined by Zhang and Chen3 using cryo-electron microscopy.

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  1. Bob Ford is at the School of Biological Sciences, The University of Manchester, Manchester, UK.,

    Bob Ford

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  1. Bob Ford
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Correspondence to Bob Ford.

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Competing interests

B.F. consulted in 2015 and 2016 for Vertex, Inc., which discovered and currently markets ivacaftor.

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Ford, B. CFTR structure: lassoing cystic fibrosis. Nat Struct Mol Biol 24, 13–14 (2017). https://doi.org/10.1038/nsmb.3353

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