The early development of ECM remodeling in PAH and the positive feedback loop connecting the ECM to the YAP/TAZ-miR-130/301 circuit suggest the pathogenic relevance of this axis both early and late in disease as well as present a model of self-sustained propagation of ECM remodeling throughout the pulmonary vascular tree as PAH progresses. The gene discussed is WWTR1; the disease is pulmonary arterial hypertension.