The vascular remodeling of pulmonary vessels documented here is also consistent with increased pulmonary vascular resistance and reduced filling of small arteries in caveolin-1 knockout animals (Maniatis et al. 2008) and with PAH-induced vascular remodeling in humans with mutations in the CAV1 gene (Austin et al. 2012). Here, CAV1 is linked to pulmonary arterial hypertension.