While the significance of this interaction is unknown, it is known that ADAMTS1 is triplicated and upregulated in DS, both in blood datasets and in DS lung13,29,39,42,76, where it contributes to the global anti-angiogenic milieu leading to higher risk for developing pulmonary arterial hypertension (PAH) in infants with DS. Here, ADAMTS1 is linked to pulmonary arterial hypertension.