Evidence for right ventricular lipotoxicity in heritable pulmonary arterial hypertension.

Hemnes AR, Brittain EL, Trammell AW, Fessel JP, Austin ED, Penner N, Maynard KB, Gleaves L, Talati M, Absi T, Disalvo T, West J
Am J Respir Crit Care Med. 2014 189 (3): 325-34

PMID: 24274756 · PMCID: PMC3977729 · DOI:10.1164/rccm.201306-1086OC

Shorter survival in heritable pulmonary arterial hypertension (HPAH), often due to BMPR2 mutation, has been described in association with impaired right ventricle (RV) compensation. HPAH animal models are insulin resistant, and cells with BMPR2 mutation have impaired fatty acid oxidation, but whether these findings affect the RV in HPAH is unknown.

MeSH Terms (17)

Animals Biomarkers Bone Morphogenetic Protein Receptors, Type II Case-Control Studies Ceramides Familial Primary Pulmonary Hypertension Genetic Markers Humans Hypertension, Pulmonary Hypertrophy, Right Ventricular Lipolysis Mice Mice, Transgenic Mutation Oligonucleotide Array Sequence Analysis Oxidation-Reduction Triglycerides

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