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.
"Our lab focus is on how vascular inflammation contributes to lung and lymphatic disease and to develop targeted therapeutics that can safely treat these conditions."
- Mark Nicolls, MD.