Macrophage dysfunction in cystic fibrosis: Nature or nurture?

Journal of Leukocyte Biology
Keren B TurtonMiguel A Valvano

Abstract

Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) affect the homeostasis of chloride flux by epithelial cells. This has deleterious consequences, especially in respiratory epithelia, where the defect results in mucus accumulation distinctive of cystic fibrosis. CFTR is, however, also expressed in phagocytic cells, like macrophages. Immune cells are highly sensitive to conditioning by their environment; thus, CFTR dysfunction in epithelia influences macrophages by affecting the lung milieu, but the mutations also appear to be directly consequential for intrinsic macrophage functions. Particular mutations can alter CFTR's folding, traffic of the protein to the membrane and function. As such, understanding the intrinsic effects of CFTR mutation requires distinguishing the secondary effects of misfolded CFTR on cell stress pathways from the primary defect of CFTR dysfunction/absence. Investigations into CFTR's role in macrophages have exploited various models, each with their own advantages and limitations. This review summarizes these methodologic approaches, discussing their physiological correspondence and highlighting key findings. The controversy surrounding CFTR-dependent acidification is used as a c...Continue Reading

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Citations

Jan 21, 2021·American Journal of Physiology. Lung Cellular and Molecular Physiology·Katja KoeppenBruce A Stanton
May 1, 2021·International Journal of Environmental Research and Public Health·Ersilia NigroAurora Daniele
Aug 17, 2021·Frontiers in Endocrinology·Lise CoderreSylvie Lesage

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