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Article

CFTR transmembrane segments are impaired in their conformational adaptability by a pathogenic loop mutation and dynamically stabilized by Lumacaftor.

The Journal of biological chemistry - 14 Feb 2020

Krainer Georg, Schenkel Mathias, Hartmann Andreas, Ravamehr-Lake Dorna, Deber Charles M, Schlierf Michael

Abstract excerpt

The cystic fibrosis transmembrane conductance regulator (CFTR) is an ion channel protein that is defective in individuals with cystic fibrosis (CF). To advance the rational design of CF therapies, it is important to elucidate how mutational defects in CFTR lead to its impairment and how pharmacological compounds interact with and alter CFTR. Here, using a helical-hairpin construct derived from CFTR's...

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Identifiers

PMID: 31882543
DOI: 10.1074/jbc.AC119.011360
PMCID: PMC7029128

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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