BRCC3 regulates the BMP signaling pathway and maintains pulmonary vascular homeostasis by deubiquitinating K472 and K475 sites of ALK2.BRCC3 deficiency exacerbates pulmonary arterial hypertension, and PPARγ agonists can partially alleviate its effects. This evidence concerns the gene ACVR1 and pulmonary arterial hypertension.