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 CFTRtargeted drug efficacy.

Item Type:	Refereed Article
Keywords:	cystic fibrosis, dendritic cells, monocytes, myeloid-derived suppressor cells, natural killer cells, peripheral blood
Research Division:	Biomedical and Clinical Sciences
Research Group:	Immunology
Research Field:	Innate immunity
Objective Division:	Health
Objective Group:	Clinical health
Objective Field:	Clinical health not elsewhere classified
UTAS Author:	Mulcahy, EM (Dr Emily Mulcahy)
UTAS Author:	Cooley, MA (Associate Professor Margaret Cooley)
UTAS Author:	Beggs, SA (Dr Sean Beggs)
UTAS Author:	Roddam, LF (Dr Louise Roddam)
ID Code:	130825
Year Published:	2019
Web of Science® Times Cited:	1
Deposited By:	Medicine
Deposited On:	2019-02-14
Last Modified:	2020-02-21
Downloads:	0