Next Article in Journal
A Terrestrial Microbial Fuel Cell for Powering a Single-Hop Wireless Sensor Network
Next Article in Special Issue
Role of the Renin-Angiotensin-Aldosterone System beyond Blood Pressure Regulation: Molecular and Cellular Mechanisms Involved in End-Organ Damage during Arterial Hypertension
Previous Article in Journal
The Clinical Significance of the Insulin-Like Growth Factor-1 Receptor Polymorphism in Non-Small-Cell Lung Cancer with Epidermal Growth Factor Receptor Mutation
Previous Article in Special Issue
Heart Disease in Women: Unappreciated Challenges, GPER as a New Target
Article Menu
Issue 5 (May) cover image

Export Article

Open AccessReview
Int. J. Mol. Sci. 2016, 17(5), 761;

Molecular Mechanisms of Pulmonary Vascular Remodeling in Pulmonary Arterial Hypertension

Division of Cardiovascular Medicine, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA 02115, USA
Division of Cardiology, Veterans Affairs Boston Healthcare System, Boston, MA 02132, USA
Author to whom correspondence should be addressed.
Academic Editor: Anastasia Susie Mihailidou
Received: 7 March 2016 / Revised: 1 April 2016 / Accepted: 8 April 2016 / Published: 18 May 2016
(This article belongs to the Special Issue Molecular Research on Hypertension)
Full-Text   |   PDF [809 KB, uploaded 18 May 2016]   |  


Pulmonary arterial hypertension (PAH) is a devastating disease that is precipitated by hypertrophic pulmonary vascular remodeling of distal arterioles to increase pulmonary artery pressure and pulmonary vascular resistance in the absence of left heart, lung parenchymal, or thromboembolic disease. Despite available medical therapy, pulmonary artery remodeling and its attendant hemodynamic consequences result in right ventricular dysfunction, failure, and early death. To limit morbidity and mortality, attention has focused on identifying the cellular and molecular mechanisms underlying aberrant pulmonary artery remodeling to identify pathways for intervention. While there is a well-recognized heritable genetic component to PAH, there is also evidence of other genetic perturbations, including pulmonary vascular cell DNA damage, activation of the DNA damage response, and variations in microRNA expression. These findings likely contribute, in part, to dysregulation of proliferation and apoptosis signaling pathways akin to what is observed in cancer; changes in cellular metabolism, metabolic flux, and mitochondrial function; and endothelial-to-mesenchymal transition as key signaling pathways that promote pulmonary vascular remodeling. This review will highlight recent advances in the field with an emphasis on the aforementioned molecular mechanisms as contributors to the pulmonary vascular disease pathophenotype. View Full-Text
Keywords: pulmonary arterial hypertension; DNA damage; microRNA; metabolism; mitochondria; endothelial-to-mesenchymal transition pulmonary arterial hypertension; DNA damage; microRNA; metabolism; mitochondria; endothelial-to-mesenchymal transition

Figure 1

This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited (CC BY 4.0).

Share & Cite This Article

MDPI and ACS Style

Leopold, J.A.; Maron, B.A. Molecular Mechanisms of Pulmonary Vascular Remodeling in Pulmonary Arterial Hypertension. Int. J. Mol. Sci. 2016, 17, 761.

Show more citation formats Show less citations formats

Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.

Related Articles

Article Metrics

Article Access Statistics



[Return to top]
Int. J. Mol. Sci. EISSN 1422-0067 Published by MDPI AG, Basel, Switzerland RSS E-Mail Table of Contents Alert
Back to Top