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Article

Combination potentiator ('co-potentiator') therapy for CF caused by CFTR mutants, including N1303K, that are poorly responsive to single potentiators.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society - 1 Sept 2018

Phuan Puay-Wah, Son Jung-Ho, Tan Joseph-Anthony, Li Clarabella, Musante Ilaria, Zlock Lorna, Nielson Dennis W, Finkbeiner Walter E, Kurth Mark J, Galietta Luis J, Haggie Peter M, Verkman Alan S

Abstract excerpt

BACKGROUND: Current modulator therapies for some cystic fibrosis-causing CFTR mutants, including N1303K, have limited efficacy. We provide evidence here to support combination potentiator (co-potentiator) therapy for mutant CFTRs that are poorly responsive to single potentiators. METHODS: Functional synergy screens done on N1303K and W1282X CFTR, in which small molecules were tested with VX-770, identified...

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

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Source

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Identifiers

PMID: 29903467
DOI: 10.1016/j.jcf.2018.05.010
PMCID: PMC6436558

Genes

CFTR

Variants

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence. Graph snapshot: 2026-09-29.

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