TNFα drives pulmonary arterial hypertension by suppressing the BMP type-II receptor and altering NOTCH signalling

Abstract excerpt

Heterozygous germ-line mutations in the bone morphogenetic protein type-II receptor (BMPR-II) gene underlie heritable pulmonary arterial hypertension (HPAH). Although inflammation promotes PAH, the mechanisms by which inflammation and BMPR-II dysfunction conspire to cause disease remain unknown. Here we identify that tumour necrosis factor-α (TNFα) selectively reduces BMPR-II transcription and mediates...

Topics

Share this publication in a Topic to start or enrich a Post.

• Share to Connective tissue disorders research
• Share to Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
• Share to Pulmonary Hypertension Research and Treatments