Does Bmpr2 mutation induce right ventricular lipotoxicity and alter stress responses in models of heritable pulmonary arterial hypertension?
BMPR2 mutations contribute to right ventricular lipotoxicity and impaired stress responses in heritable pulmonary arterial hypertension, highlighting a potential metabolic mechanism for RV dysfunction.
These data demonstrate that Bmpr2 mutation affects RV stress responses in a transgenic rodent model. Impaired RV hypertrophy and triglyceride and ceramide deposition are present as a function of RV mutant Bmpr2 in mice; fatty acid oxidation impairment in human HPAH RVs may underlie this finding. Further study of how BMPR2 mediates RV lipotoxicity is warranted.
Hemnes et al. (Mon,) studied this question.
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