Pulmonary arterial hypertension (PAH) is a rare, progressive disorder typified by occlusion
of the pulmonary arterioles owing to endothelial dysfunction and uncontrolled proliferation of …

The unique architecture of the mammalian lung is required for adaptation to air breathing at
birth and thereafter. Understanding the cellular and molecular mechanisms controlling its …

Pulmonary arterial hypertension (PAH) is a rare disorder with a poor prognosis. Deleterious
variation within components of the transforming growth factor-β pathway, particularly the …

Background Rare genetic variants cause pulmonary arterial hypertension, but the
contribution of common genetic variation to disease risk and natural history is poorly …

Alveolarization is the process by which the alveoli, the principal gas exchange units of the
lung, are formed. Along with the maturation of the pulmonary vasculature, alveolarization is …

Background Pulmonary arterial hypertension (PAH) is a rare disease characterized by
distinctive changes in pulmonary arterioles that lead to progressive pulmonary arterial …

The Hippo–YAP1/TAZ pathway is a highly conserved central mechanism that controls organ
size through the regulation of cell proliferation and other physical attributes of cells. The …

Each of the steps of respiratory system development relies on intricate interactions and
coordinated development of the lung epithelium and mesenchyme. In the past, more …

Idiopathic pulmonary fibrosis (IPF) is a progressive, irreversible fibrotic disease of the distal
lung alveoli that culminates in respiratory failure and reduced lifespan. Unlike normal lung …

Background The phenotype of pulmonary arterial hypertension (PAH) patients carrying
SOX17 pathogenic variants remains mostly unknown. Methods We report the genetic …