Endothelial-to-mesenchymal transition in pulmonary hypertension

Abstract

Pulmonary arterial hypertension (PAH) is an incurable disease characterized by an intense thickening of the pulmonary arteries leading to their progressive obliteration. This vascular remodeling is a direct consequence of an aberrant accumulation of α-smooth muscle actin (α-SMA)-positive cells in the intravascular area. This process is multifactorial and complex. Recent studies have demonstrated that part of α-SMA-positive cells from intimal and plexiform lesions have an endothelial origin through a process called endothelial-to-mesenchymal transition (EndoMT). Interestingly most of molecular pathways implicated in PAH are known inducers of EndoMT. This review describes how EndoMT appears to play a crucial role in PAH progression.