Abstract
Pulmonary arterial hypertension (PAH) is a vascular remodeling disease with a relentless course toward heart failure and ultimately death [1-3]. Although existing therapies improve patients’ quality of life and symptoms, none of the currently approved therapies can reverse the disease or significantly reduce mortality. The treatment is also extremely expensive and often associated with side effects. As such, furthering our understanding of the mechanisms and pathology of PAH is important to support more effective therapeutic target identification.
Highlights
The importance of BMPR2 in pulmonary hypertension has been supported by several animal studies
SMAD1 is one of the canonical signal transducers of the BMPR2 pathway, and its reduced activity has been shown to be associated with Pulmonary arterial hypertension (PAH)
Their study shows that the BMPR2-deficient endothelial cells demonstrate the presence of an opposing balance between TGF-β1 and BMP4 signaling in endothelial cells, suggesting that diminished SMAD1 signaling and enhanced TGF-β signaling may contribute to PAH development
Summary
The importance of BMPR2 in pulmonary hypertension has been supported by several animal studies. SMAD1 is one of the canonical signal transducers of the BMPR2 pathway, and its reduced activity has been shown to be associated with PAH. Han et al [14] have investigated a cell-type specific role of SMAD1 in PAH pathogenesis by conditionally deleting the Smad1 gene in either endothelial cells or in smooth muscle cells using an L1Cre or Taglin-Cre line and demonstrating that SMAD1 is a critical downstream molecule of the BMPR2 pathway in PAH.
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