Bone Morphogenetic Protein 9 Is a Mechanistic Biomarker of Portopulmonary Hypertension.

ABSTRACT:

Rationale

BMP9 (bone morphogenetic protein 9) is a circulating endothelial quiescence factor with protective effects in pulmonary arterial hypertension (PAH). Loss-of-function mutations in BMP9, its receptors, and downstream effectors have been reported in heritable PAH.

Objectives

To determine how an acquired deficiency of BMP9 signaling might contribute to PAH.

Methods

Plasma levels of BMP9 and antagonist soluble endoglin were measured in group 1 PAH, group 2 and 3 pulmonary hypertension (PH), and in patients with severe liver disease without PAH.

Measurements and main results

BMP9 levels were markedly lower in portopulmonary hypertension (PoPH) versus healthy control subjects, or other etiologies of PAH or PH; distinguished PoPH from patients with liver dise

SUBMITTER: Nikolic I 

PROVIDER: S-EPMC6444661 | biostudies-literature | 2019 Apr

REPOSITORIES: biostudies-literature