Abstract
Pulmonary hypertension (PH) is a rare but severe disease. Endothelial and vascular smooth muscle cells are critically involved in the pathology of PH. However, pulmonary vessels are not accessible in most patients. Blood outgrowth (BO) vascular cells are derived from progenitors and are potentially valuable, highly translatable models. Here, we show for the first time that endothelial cells (BOEC) and putative smooth muscle cells can be grown out from the blood of patients with PH. BOEC from patients (n=2) aligned under complex shear conditions in a way characteristic of endothelial cells, expressed VE-cadherin and released endothelin-1. Autologous vascular smooth muscle cells were isolated from the same patients’ peripheral blood samples and displayed a typical ‘hill and valley’ morphology (Fig A). As interferon (IFN) signalling is thought to be relevant to vascular dysfunction, responses of BOEC from PH patients (n=3) to IFNα and IFNγ (30ng/ml) were compared to healthy donor cells (n=4-6). IP10 (CXCL10) was measured as a ubiquitous readout of IFN signalling. BOEC from PH patients released more IP10 in response to IFNs (Fig B). The ability to derive both endothelial and smooth muscle cells from individual patients with PH represents a key step forward in translational and personalised research in this condition.
This study was supported by an unconditional educational award from Actelion.
- © 2013 ERS
