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Article

VX-770-mediated potentiation of numerous human CFTR disease mutants is influenced by phosphorylation level.

Scientific reports - 17 Sept 2019

Cui Guiying, Stauffer Brandon B, Imhoff Barry R, Rab Andras, Hong Jeong S, Sorscher Eric J, McCarty Nael A

Abstract excerpt

VX-770 (ivacaftor) is approved for clinical use in CF patients bearing multiple CFTR mutations. VX-770 potentiated wildtype CFTR and several disease mutants expressed in oocytes in a manner modulated by PKA-mediated phosphorylation. Potentiation of some other mutants, including G551D-CFTR, was less dependent upon the level of phosphorylation, likely related to the severe gating defects in these mutants exhibited...

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Source

Open in PubMed Open full text

Identifiers

PMID: 31530897
DOI: 10.1038/s41598-019-49921-4
PMCID: PMC6749054

Genes

CFTR

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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