Heterozygous null bone morphogenetic protein receptor type 2 mutations promote SRC kinase-dependent caveolar trafficking defects and endothelial dysfunction in pulmonary arterial hypertension.

@article{Prewitt2015HeterozygousNB,
  title={Heterozygous null bone morphogenetic protein receptor type 2 mutations promote SRC kinase-dependent caveolar trafficking defects and endothelial dysfunction in pulmonary arterial hypertension.},
  author={Allison R Prewitt and Sampa Ghose and Andrea L. Frump and Arumima Datta and Eric D Austin and Anne K. Kenworthy and Mark P. de Caestecker},
  journal={The Journal of biological chemistry},
  year={2015},
  volume={290 2},
  pages={960-71}
}
Hereditary pulmonary arterial hypertension (HPAH) is a rare, fatal disease of the pulmonary vasculature. The majority of HPAH patients inherit mutations in the bone morphogenetic protein type 2 receptor gene (BMPR2), but how these promote pulmonary vascular disease is unclear. HPAH patients have features of pulmonary endothelial cell (PEC) dysfunction including increased vascular permeability and perivascular inflammation associated with decreased PEC barrier function. Recently, frameshift… CONTINUE READING
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