In a model of hypoxia-induced PAH, mice bearing a targeted deletion of Adamts8 in pulmonary arterial smooth muscle cells (Adamts8ΔSM22α) showed decreased right ventricular systolic pressure and right ventricular hypertrophy compared with wild-type mice, suggesting a crucial role for ADAMTS-8 in the development of PAH [119]. Here, ADAMTS8 is linked to pulmonary arterial hypertension.