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Widespread alterations in the peripheral blood innate immune cell profile in cystic fibrosis reflect lung pathology
Author(s) -
Mulcahy Emily M,
Cooley Margaret A,
McGuire Helen,
Asad Suzanne,
Fazekas de St Groth Barbara,
Beggs Sean A,
Roddam Louise F
Publication year - 2019
Publication title -
immunology and cell biology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.999
H-Index - 104
eISSN - 1440-1711
pISSN - 0818-9641
DOI - 10.1111/imcb.12230
Subject(s) - immune system , innate immune system , immunology , cystic fibrosis , biology , cystic fibrosis transmembrane conductance regulator , monocyte , phenotype , cell , innate lymphoid cell , gene , biochemistry , genetics
Cystic fibrosis ( CF ) is caused by mutations to the CF transmembrane conductance regulator ( CFTR ) gene. CFTR is known to be expressed on multiple immune cell subtypes, dendritic cells, monocytes/macrophages, neutrophils and lymphocytes. We hypothesized that the lack of CFTR expression on peripheral blood innate immune cells would result in an altered cell profile in the periphery and that this profile would reflect lung pathology. We performed a flow cytometric phenotypic investigation of innate immune cell proportions in peripheral blood collected from 17 CF patients and 15 age‐matched healthy controls. We observed significant differences between CF patients and controls in the relative proportions of natural killer ( NK ) cells, monocytes and their subsets, with significant correlations observed between proportions of NK and monocyte cell subsets and lung function (forced expiratory volume in 1 sec, % predicted; FEV1% predicted) in CF patients. This study demonstrates the widespread nature of immune dysregulation in CF and provides a basis for identification of potential therapeutic targets. Modulation of the distinct CF ‐related immune cell phenotype identified could also be an important biomarker for evaluating CFTR ‐targeted drug efficacy.