Targeting the TGF-ß signaling pathway for resolution of pulmonary arterial hypertension.

Aberrant transforming growth factor-ß (TGF-ß) signaling activation is linked to pulmonary arterial hypertension (PAH). BMPR2 mutations perturb the balance between bone morphogenetic protein (BMP) and TGF-ß pathways, leading to vascular remodeling, narrowing of the lumen of pulmonary vasculature, and clinical symptoms. This forum highlights the association of the TGF-ß pathway with pathogenesis and therapeutic approaches.