Bone Morphogenetic Protein 9 Is a Mechanistic Biomarker of Portopulmonary Hypertension.
Ivana NikolicLai-Ming YungPeiran YangRajeev MalhotraSamuel D Paskin-FlerlageTeresa DinterGeoffrey A BocoboKathleen E TumeltyAnthony J FaugnoLuca TronconeMegan E McNeilXiuli HuangKathryn R CoserCarol S C LaiPaul D UptonMarie-José T H GoumansRoham T ZamanianC Gregory ElliottArthur LeeWei ZhengStephen P BerasiChristine HuardNicholas W MorrellRaymond T ChungRichard W ChannickKari E RobertsPaul B YuPublished in: American journal of respiratory and critical care medicine (2020)
BMP9 is a sensitive and specific biomarker of PoPH, predicting transplant-free survival and the presence of PAH in liver disease. In rodent models, acquired deficiency of BMP9 signaling can predispose to or exacerbate PH, providing a possible mechanistic link between PoPH and heritable PAH. These findings describe a novel experimental model of severe PH that provides insight into the synergy between pulmonary vascular injury and diminished BMP9 signaling in the pathogenesis of PAH.