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Widespread alterations in the peripheral blood innate immune cell profile in cystic fibrosis reflect lung pathology
Citation
Mulcahy, EM and Cooley, MA and McGuire, H and Asad, S and Fazekas de St Groth, B and Beggs, SA and Roddam, LF, Widespread alterations in the peripheral blood innate immune cell profile in cystic fibrosis reflect lung pathology, Immunology and Cell Biology pp. 1-11. ISSN 0818-9641 (2019) [Refereed Article]
Copyright Statement
Copyright 2019 Australasian Society for Immunology Inc.
Abstract
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 Details
Item Type: | Refereed Article |
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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 |
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