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

ABSTRACT:

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 were analyzed for inflammatory and growth-related proteins at baseline, 1 and 6 months after ivacaftor initiation. In 30 subjects, plasma was assayed for 1,322 proteins using the SomaScan proteomic platform at baseline and 6 months post-ivacaftor. Correlations with clinical outcomes were assessed.

Measurements and main results

Significant reductions in high mob

SUBMITTER: Hoppe JE 

PROVIDER: S-EPMC9569394 | biostudies-literature | 2022 Nov

REPOSITORIES: biostudies-literature