Abstract 18780: Down-regulation of BMPR2 in Pulmonary Hypertension Secondary to Lung Fibrosis

Pulmonary Hypertension (PH) is a disorder affecting the vasculature of the lung. A highly prevalent form of PH is that associated with underlying chronic lung diseases such as chronic obstructive pulmonary disease (COPD) or idiopathic pulmonary fibrosis (IPF). However, little is known about the mechanisms that drive the development of PH secondary to chronic lung diseases. Loss of function mutations in bone morphogenic protein receptor (BMPR) 2 have been strongly linked to familial and non-familial cases of pulmonary arterial hypertension (PAH). In PAH, loss of function BMPR2 mutations lead to pronounced vascular remodeling and increased responsiveness of vascular smooth muscle cells to the pro-fibrotic cytokine, transforming growth factor (TGF)-β. In addition, the loss of BMPR2 function in vascular smooth muscle cells abrogates the anti-proliferative effects of BMP ligands by altering downstream Smad signaling pathways. Our hypothesis is that BMPR2 is down-regulated in PH secondary to lung fibrosis. Usin...