PT  - JOURNAL ARTICLE
AU  - Vasile Foris
AU  - Gabor Kovacs
AU  - Leigh M. Marsh
AU  - Zoltán Bálint
AU  - Martin Tötsch
AU  - Alexander Avian
AU  - Philipp Douschan
AU  - Bahil Ghanim
AU  - Walter Klepetko
AU  - Andrea Olschewski
AU  - Horst Olschewski
TI  - CD133<sup>+</sup> cells in pulmonary arterial hypertension
AID  - 10.1183/13993003.01523-2015
DP  - 2016 Aug 01
TA  - European Respiratory Journal
PG  - 459--469
VI  - 48
IP  - 2
4099  - http://erj.ersjournals.com/content/48/2/459.short
4100  - http://erj.ersjournals.com/content/48/2/459.full
SO  - Eur Respir J2016 Aug 01; 48
AB  - 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.PAH patients are characterised by lymphopenia and increased numbers of CD133+ cells http://ow.ly/YNfQE