Restoring BMPRII functions in pulmonary arterial hypertension: opportunities, challenges and limitations.

Abstract excerpt

INTRODUCTION: Pulmonary arterial hypertension (PAH) is a cardiopulmonary disorder in which mechanical obstruction of the pulmonary vascular bed is largely responsible for the rise in pulmonary arterial pressures. The discovery of heterozygous BMPR2 germline mutations as critical predisposing factors together with a remarkable progress in our understanding of the pathogenic mechanisms have helped identify the...

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