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Article

Effects of ivacaftor on systemic inflammation and the plasma proteome in people with CF and G551D.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society - 1 Nov 2022

Hoppe Jordana E, Wagner Brandie D, Kirk Harris J, Rowe Steven M, Heltshe Sonya L, DeBoer Emily M, Sagel Scott D

Abstract excerpt

BACKGROUND: Ivacaftor is a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator for people with CF and the G551D mutation. We aimed to investigate the biology of CFTR modulation and systemic effects of CFTR restoration by examining changes in circulating measurements of inflammation and growth and novel proteins with ivacaftor treatment. METHODS: Blood samples from 64 CF subjects with G551D-CFTR...

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Identifiers

PMID: 35440409
DOI: 10.1016/j.jcf.2022.03.012
PMCID: PMC9569394

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