PT - JOURNAL ARTICLE AU - Olga Tura-Ceide AU - Núria Aventín-Beamonte AU - Núria Chamorro-Tort AU - Jéssica García-Lucio AU - Núria Coll-Bonfill AU - Isabel Blanco-Vich AU - Víctor, Ivo Peinado Cabré AU - José Luís Pomar AU - Manel Catesllà Pericàs AU - Joan Albert Barberà Mir TI - Derivation and characterisation of endothelial cells from patients with chronic thromboembolic pulmonary hypertension DP - 2014 Sep 01 TA - European Respiratory Journal PG - P2327 VI - 44 IP - Suppl 58 4099 - http://erj.ersjournals.com/content/44/Suppl_58/P2327.short 4100 - http://erj.ersjournals.com/content/44/Suppl_58/P2327.full SO - Eur Respir J2014 Sep 01; 44 AB - Rationale:Pulmonary endarterectomy (PEA) is the gold standard treatment for Chronic Thromboembolic Pulmonary Hypertension (CTEPH). This material provides a unique opportunity to study the pathophysiological mechanisms of CTEPH at disease site. Endothelial dysfunction might be an important step in the development of CTEPH. In this study, we aimed to develop an in vitro model of CTEPH endothelial pathology using patient-derived cell lines and to determine whether such cells are dysfunctional or present an impaired angiogenic potential.Methods:Isolated cells from freshly resected specimens obtained at PEA, were confirmed as being endothelial cells based on cobblestone morphology, endothelial phenotype (flow cytometry, RT-PCR, immunofluorescence) and functional analysis (tubule formation, proliferation and migration).Results: Isolated cells maintained cobblestone morphology and stained positive for endothelial markers: CD31, CD146, CD34, CD144, VWF, UEA-1, eNOS; and negative for CD45, SMA, CD56 and calponin. They showed a hyperproliferative phenotype when compared with control human pulmonary artery endothelial cell lines (HPAE): number of Ki67+cells (50.33±13.4 vs 32.5±9.5; p<0.05), and fold expansion (1.56±0.08 vs 0.8±0.05; p<0.002). Functionally, they showed reduced capacity to migrate (p<0.02) and to form tubule structures (150±44 vs 96±21; p<0.03).Conclusions:This study provides a novel endothelial cell line obtained from PEA material that has the potential to provide new insight into the role of endothelial dysfunction in CTEPH.OTC is supported by BIOTRACK: IDIBAPS Postdoctoral Programme and Garcia-Lucio is recipient of PFIS grant from ISCIII.