Mechanisms of Lipid Accumulation in the Bone Morphogenic Protein Receptor 2 Mutant Right Ventricle.

Abstract

In heritable pulmonary arterial hypertension (HPAH) with germline mutation in the bone morphogenetic protein receptor 2 (BMPR2) gene, right ventricular (RV) dysfunction is associated with RV lipotoxicity, however the underlying mechanism for lipid accumulation is not known.