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Ranchoux B, Harvey LD, Ayon RJ, et al. Endothelial dysfunction in pulmonary arterial hypertension: an evolving landscape (2017 Grover Conference Series). Pulm Circ. 2017;8(1):2045893217752912doi: 10.1177/2045893217752912.
Ranchoux, B., Harvey, L. D., Ayon, R. J., Babicheva, A., Bonnet, S., Chan, S. Y., Yuan, J. X., & Perez, V. J. (2018). Endothelial dysfunction in pulmonary arterial hypertension: an evolving landscape (2017 Grover Conference Series). Pulmonary circulation, 8(1), 2045893217752912. https://doi.org/10.1177/2045893217752912
Ranchoux, Benoît, et al. "Endothelial dysfunction in pulmonary arterial hypertension: an evolving landscape (2017 Grover Conference Series)." Pulmonary circulation vol. 8,1 (2018): 2045893217752912. doi: https://doi.org/10.1177/2045893217752912
Ranchoux B, Harvey LD, Ayon RJ, Babicheva A, Bonnet S, Chan SY, Yuan JX, Perez VJ. Endothelial dysfunction in pulmonary arterial hypertension: an evolving landscape (2017 Grover Conference Series). Pulm Circ. 2018 Jan-Mar;8(1):2045893217752912. doi: 10.1177/2045893217752912. Epub 2017 Dec 28. PMID: 29283043; PMCID: PMC5798691.
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Pulm Circ. 2018 Jan-Mar;8(1):2045893217752912. doi: 10.1177/2045893217752912. Epub 2017 Dec 28.

Endothelial dysfunction in pulmonary arterial hypertension: an evolving landscape (2017 Grover Conference Series).

Pulmonary circulation

Benoît Ranchoux, Lloyd D Harvey, Ramon J Ayon, Aleksandra Babicheva, Sebastien Bonnet, Stephen Y Chan, Jason X-J Yuan, Vinicio de Jesus Perez

Affiliations

  1. 1 55973 Centre de Recherche de l'IUCPQ , Quebec Canada.
  2. 2 University of Pittsburgh Vascular Medicine Institute Division of Cardiology, Pittsburgh, PA, USA.
  3. 3 8041 Division of Translational and Regenerative Medicine , The University of Arizona College of Medicine, Tucson, AZ, USA.
  4. 4 Division of Pulmonary and Critical Care Medicine, Stanford University Medical Center, Stanford, CA, USA.
  5. 5 The Vera Moulton Wall Center for Pulmonary Vascular Medicine, Stanford University Medical Center, Stanford, CA, USA.
  6. 6 Stanford Cardiovascular Institute, Stanford University Medical Center, Stanford, CA, USA.

PMID: 29283043 PMCID: PMC5798691 DOI: 10.1177/2045893217752912

Abstract

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.

Keywords: angiogenesis; endothelial to mesenchymal transition; endothelium; epigenetics; voltage-gated ion channels

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