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Article

Proteostasis landscapes of cystic fibrosis variants reveal drug response vulnerability.

Proceedings of the National Academy of Sciences of the United States of America - 29 Apr 2025

McDonald Eli Fritz, Kim Minsoo, Olson John A, Meiler Jens, Plate Lars

Abstract excerpt

Cystic fibrosis (CF) is a lethal genetic disorder caused by variants in CF transmembrane conductance regulator (CFTR). Many variants are treatable with correctors, which enhance the folding and trafficking of CFTR. However, approximately 3% of persons with CF harbor poorly responsive variants. Here, we used affinity purification mass spectrometry proteomics to profile the protein homeostasis (proteostasis)...

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Topics

Cystic Fibrosis Transmembrane Conductance Regulator
Cystic Fibrosis
Humans
Proteostasis
Protein Folding
Mutation

Methodology Data sources Editorial policy Privacy Delete account & data About Contact Helena Bioinformatics

Benzodioxoles

Proteomics

Aminopyridines

Source

Open in PubMed Open full text

Identifiers

PMID: 40261935
DOI: 10.1073/pnas.2418407122
PMCID: PMC12054793

Genes

CFTR

Provenance

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

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