The Sugen 5416/Hypoxia Mouse Model of Pulmonary Arterial Hypertension

: Pulmonary hypertension is a rapidly progressive, life-threatening, and often fatal disease. Despite many new developments in pulmonary arterial hypertension (PAH) therapy, there is currently no cure for PAH, and new therapies are desperately needed. PAH pathobiology involves a remodeling process in pulmonary arteries that plays a critical role in elevating pulmonary arterial and right ventricle pressures. The discovery and development of new therapies requires animal models of PAH that mimic the human disease, including vascular remodeling.Here we review and describe a detailed protocol for creating an in vivo model of Sugen/Hypoxia-induced PAH in mice that is commonly used to assess the efficiency of new therapies in PAH. Severe pulmonary hypertension can be established in 1 month using this protocol. Additional protocols to evaluate the model by invasive pressure measurements and histology are provided.