TY - JOUR
T1 - Endothelial dysfunction in pulmonary arterial hypertension
T2 - An evolving landscape (2017 Grover Conference Series)
AU - Ranchoux, Benoît
AU - Harvey, Lloyd D.
AU - Ayon, Ramon J.
AU - Babicheva, Aleksandra
AU - Bonnet, Sebastien
AU - Chan, Stephen Y.
AU - Yuan, Jason X.J.
AU - Perez, Vinicio de Jesus
N1 - Funding Information:
This work was supported by National Institute of Health grants HL096834, HL124021, HL138437, and TR002073 (to SYC).
Publisher Copyright:
© The Author(s) 2018.
PY - 2018/1/1
Y1 - 2018/1/1
N2 - Endothelial dysfunction is a major player in the development and progression of vascular pathology in pulmonary arterial hypertension (PAH), a disease associated with small vessel loss and obstructive vasculopathy that leads to increased pulmonary vascular resistance, subsequent right heart failure, and premature death. Over the past ten years, there has been tremendous progress in our understanding of pulmonary endothelial biology as it pertains to the genetic and molecular mechanisms that orchestrate the endothelial response to direct or indirect injury, and how their dysregulation can contribute to the pathogenesis of PAH. As one of the major topics included in the 2017 Grover Conference Series, discussion centered on recent developments in four areas of pulmonary endothelial biology: (1) angiogenesis; (2) endothelial-mesenchymal transition (EndMT); (3) epigenetics; and (4) biology of voltage-gated ion channels. The present review will summarize the content of these discussions and provide a perspective on the most promising aspects of endothelial dysfunction that may be amenable for therapeutic development.
AB - Endothelial dysfunction is a major player in the development and progression of vascular pathology in pulmonary arterial hypertension (PAH), a disease associated with small vessel loss and obstructive vasculopathy that leads to increased pulmonary vascular resistance, subsequent right heart failure, and premature death. Over the past ten years, there has been tremendous progress in our understanding of pulmonary endothelial biology as it pertains to the genetic and molecular mechanisms that orchestrate the endothelial response to direct or indirect injury, and how their dysregulation can contribute to the pathogenesis of PAH. As one of the major topics included in the 2017 Grover Conference Series, discussion centered on recent developments in four areas of pulmonary endothelial biology: (1) angiogenesis; (2) endothelial-mesenchymal transition (EndMT); (3) epigenetics; and (4) biology of voltage-gated ion channels. The present review will summarize the content of these discussions and provide a perspective on the most promising aspects of endothelial dysfunction that may be amenable for therapeutic development.
KW - Angiogenesis
KW - Endothelial to mesenchymal transition
KW - Endothelium
KW - Epigenetics
KW - Voltage-gated ion channels
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U2 - 10.1177/2045893217752912
DO - 10.1177/2045893217752912
M3 - Review article
AN - SCOPUS:85041399124
SN - 2045-8932
VL - 8
JO - Pulmonary Circulation
JF - Pulmonary Circulation
IS - 1
ER -