Circulating mononuclear cells may play an important role for the vascular remodelling in pulmonary arterial hypertension (PAH), but studies addressing multiple progenitor populations are rare and inconsistent. We used a comprehensive fluorescence-activated cell sorting analysis of circulating mononuclear cells in 20 PAH patients and 20 age-and sex-matched controls, and additionally analysed CD133+ cells in the lung tissue of five PAH transplant recipients and five healthy controls (donor lungs). PAH patients were characterised by increased numbers of circulating CD133+ cells and lymphopenia as compared with control. In PAH, CD133+ subpopulations positive for CD117 or CD45 were significantly increased, whereas CD133+CD309+, CD133+CXCR2+ and CD133+CD31+ cells were decreased. In CD133+ cells, SOX2, Nanog, Ki67 and CXCR4 were not detected, but Oct3/4 mRNA was present in both PAH and controls. In the lung tissue, CD133+ cells included three main populations: type 2 pneumocytes, monocytes and undifferentiated cells without significant differences between PAH and controls. In conclusion, circulating CD133+ progenitor cells are elevated in PAH and consist of phenotypically different subpopulations that may be up-or downregulated. This may explain the inconsistent results in the literature. CD133+ type 2 pneumocytes in the lung tissue are not associated with circulating CD133+ mononuclear cells.
CITATION STYLE
Foris, V., Kovacs, G., Marsh, L. M., Bálint, Z., Tötsch, M., Avian, A., … Olschewski, H. (2016). CD133+ cells in pulmonary arterial hypertension. European Respiratory Journal, 48(2), 459–469. https://doi.org/10.1183/13993003.01523-2015
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