Spiekerkoetter Lab


Led by Dr. Edda Spiekerkoetter, our research focuses on the importance of the Bone Morphogenetic Protein Receptor 2 (BMPR2) signaling pathway in pulmonary, pulmonary-vascular as well as cardiac disease.

In 2000 two independent groups discovered mutations in the BMPR2 pathway as the genetic basis for pulmonary arterial hypertension (PAH). Over the past years more mutations either directly involved in the BMPR2 pathway (Endoglin, Alk1, Smad9) or indirectly linked to the BMPR2 pathway (Caveolin-1) have been discovered, emphasizing the central role of BMPR2 signaling in familial PAH. It was subsequently found that reduced BMPR2 expression and signaling seems to be a feature of other sporadic or idiopathic forms of PAH.