@article {Foris2942, author = {Vasile Foris and Gabor Kovacs and Leigh Marsh and Zolt{\'a}n B{\'a}lint and Martin T{\"o}tsch and Alexander Avian and Philipp Douschan and Maria Tscherner and Andrea Olschewski and Horst Olschewski}, title = {Characterization of circulating CD133+ progenitor cells in pulmonary arterial hypertension}, volume = {44}, number = {Suppl 58}, elocation-id = {2942}, year = {2014}, publisher = {European Respiratory Society}, abstract = {BackgroundLittle is known about the relevance of circulating progenitor cells in pulmonary arterial hypertension (PAH). We aimed to characterize the circulating CD133+ cells in PAH and tested the hypothesis that CD133 may serve as biomarker.Patients and MethodsCD133+ cells were analyzed by flow cytometry from peripheral blood of n=20 patients with PAH and their age -and sex-matched controls (n=20). The number of CD133+ population was then correlated with patients{\textquoteright} haemodynamics. CD133+ cells were sorted from n=10 PAH and n=10 healthy donors by MACS. RNA was isolated and cDNA was obtained after preamplification step. RT-PCR was performed for the following genes: Oct3/4, SOX2, Nanog, Ki67 and CXRCR4. Immunohistochemistry was performed on n=5 IPAH and n=5 healthy donor lung tissues for CD133, αSMA, vWfa, Ki67 and H\&E.ResultsCD133+ cells were elevated in PAH as compared to controls (p\<0.0004). CD133+ CD14+ cells were also significantly elevated (p\<0.0001) in PAH as compared to controls. The CD133+ cells of bone marrow origin (CD133+CD45+) were also significantly increased (p\<0.0004). The number of CD133+ cells correlated with mPAP and PVR. CD133+ cells did not express SOX2, Nanog, Ki67 and CXCR4 on mRNA levels, whereas Oct3/4 mRNA was present in both PAH and healthy controls with no statistically significant difference between the two groups. Immunohistochemistry showed that CD133+ cells were present in the lung tissue and comprised: Type 2 pneumocytes, monocytic cells and undifferentiated cells.ConclusionCirculating CD133+ cells are elevated in PAH as compared to controls and their number correlates with the pulmonary hemodynamics. CD133 may serve as a potential biomarker for PAH.}, issn = {0903-1936}, URL = {https://erj.ersjournals.com/content/44/Suppl_58/2942}, eprint = {https://erj.ersjournals.com/content}, journal = {European Respiratory Journal} }