Disease Research Area 3 (Pulmonary Hypertension)

Pulmonary Hypertension is a deadly disease, currently without a cure. We discovered that the aberrant immune/inflammatory responses cause the pulmonary arterial endothelium transformation and contributes to the evolution of the occlusive arteriopathy, that is characterized by apoptosis, apoptosis resistance, hyper-proliferation and endothelial mesenchymal transition of the endothelial cells. We also found that this unique inflammatory endothelial cell phenotype is related to the dysfunction of a key genetic modified of Pulmonary Hypertension, bone morphogenetic protein receptor 2 (BMPR2).

Our research determines the relationship between a genetic vulnerability causing loss of a protective pathway related to abnormal BMPR2 function and a deleterious innate immune factors names LTB4 and evaluates how these factors contribute to the obliteration of lung vessels and to the progression of Pulmonary Hypertension.