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Article

Altered functional interactions between CFTR disease mutants ΔF508 and G551D and the protein kinase A catalytic subunit.

The Journal of physiology - 1 Apr 2026

Závoti Olivér, Simon Márton A, Csanády László

Abstract excerpt

The epithelial anion channel cystic fibrosis transmembrane conductance regulator (CFTR) is activated by cAMP-dependent protein kinase (PKA). PKA stimulates CFTR channels through two mechanisms: non-catalytically, by binding to the channel, and catalytically, by phosphorylating its regulatory (R) domain. CFTR mutations that reduce channel activity cause cystic fibrosis (CF), but clinically used modulator drugs...

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Source

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Identifiers

PMID: 41920076
DOI: 10.1113/JP290798
PMCID: PMC13082197

Genes

CFTR

Variants

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 23 Oct 2026.

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Altered functional interactions between CFTR disease mutants ΔF508 and G551D and the protein kinase A catalytic subunit.PMID 41920076Read ↗

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A small molecule CFTR potentiator restores ATP-dependent channel gating to the cystic fibrosis mutant G551D-CFTR.Semantic proximity Read ↗

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Phosphorylation of protein kinase C sites in NBD1 and the R domain control CFTR channel activation by PKA.Semantic proximity Read ↗

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