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Article

Chronic and acute modulator treatment restore wild-type-like activity and stability to the primary cystic fibrosis-causing CFTR variant.

Proceedings of the National Academy of Sciences of the United States of America - 3 Feb 2026

Rodrat Mayuree, Charlick James N, Sheppard David N

Abstract excerpt

Elexacaftor-tezacaftor-ivacaftor has transformed the treatment of people with cystic fibrosis (CF) and the predominant cystic fibrosis transmembrane conductance regulator (CFTR) variant F508del by delivering the faulty channel to the plasma membrane and potentiating its activity. However, the action of elexacaftor-tezacaftor-ivacaftor on the molecular behavior of F508del-CFTR is unknown. Here, we investigated the...

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Source

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Identifiers

PMID: 41576103
DOI: 10.1073/pnas.2523950123
PMCID: PMC12867690

Genes

CFTR

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 4 Sept 2026.

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Chronic and acute modulator treatment restore wild-type-like activity and stability to the primary cystic fibrosis-causing CFTR variant.PMID 41576103Read ↗

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