Role of extracellular matrix in the pathogenesis of pulmonary arterial hypertension

American Journal of Physiology. Heart and Circulatory Physiology
Thenappan ThenappanE Kenneth Weir

Abstract

Pulmonary arterial hypertension (PAH) is characterized by remodeling of the extracellular matrix (ECM) of the pulmonary arteries with increased collagen deposition, cross-linkage of collagen, and breakdown of elastic laminae. Extracellular matrix remodeling occurs due to an imbalance in the proteolytic enzymes, such as matrix metalloproteinases, elastases, and lysyl oxidases, and tissue inhibitor of matrix metalloproteinases, which, in turn, results from endothelial cell dysfunction, endothelial-to-mesenchymal transition, and inflammation. ECM remodeling and pulmonary vascular stiffness occur early in the disease process, before the onset of the increase in the intimal and medial thickness and pulmonary artery pressure, suggesting that the ECM is a cause rather than a consequence of distal pulmonary vascular remodeling. ECM remodeling and increased pulmonary arterial stiffness promote proliferation of pulmonary vascular cells (endothelial cells, smooth muscle cells, and adventitial fibroblasts) through mechanoactivation of various signaling pathways, including transcriptional cofactors YAP/TAZ, transforming growth factor-β, transient receptor potential channels, Toll-like receptor, and NF-κB. Inhibition of ECM remodeling and me...Continue Reading

References

Dec 1, 1991·The American Journal of Physiology·K MaruyamaM Rabinovitch
Apr 1, 1990·Circulation Research·G J PoianiD J Riley
Feb 1, 1987·The American Review of Respiratory Disease·J S KerrD J Riley
Oct 10, 2001·Clinics in Chest Medicine·M Rabinovitch
Jun 14, 2002·American Journal of Physiology. Heart and Circulatory Physiology·John E JonesYing-Yi Zhang
May 3, 2005·The European Respiratory Journal·H LepetitC Lafuma
Sep 2, 2005·Journal of the American Society of Nephrology : JASN·Kayoko MiyataAkira Nishiyama
Feb 21, 2006·Journal of the American College of Cardiology·Srijoy MahapatraMichael D McGoon
Apr 7, 2006·Journal of Bone and Mineral Research : the Official Journal of the American Society for Bone and Mineral Research·Mattabhorn PhimphilaiRenny T Franceschi
Aug 23, 2006·Cell·Adam J EnglerDennis E Discher
Mar 18, 2008·European Journal of Cell Biology·Pierre-Jean Wipff, Boris Hinz
Mar 20, 2008·European Heart Journal·Jan-Willem LankhaarAnton Vonk-Noordegraaf
Jul 22, 2008·Biomechanics and Modeling in Mechanobiology·Kristine D Estrada, Naomi C Chesler
Dec 17, 2008·Circulation Research·M Kathryn SteinerAaron B Waxman
Mar 24, 2009·Arteriosclerosis, Thrombosis, and Vascular Biology·Cindy Ying Yin YipCraig A Simmons
Apr 10, 2009·JACC. Cardiovascular Imaging·Javier SanzSanjay Rajagopalan
Jul 9, 2009·Journal of the American College of Cardiology·Paul M HassounMarc Humbert
Dec 25, 2009·The European Respiratory Journal·T ThenappanM Gomberg-Maitland
Mar 27, 2010·American Journal of Respiratory and Critical Care Medicine·Aránzazu CampoPaul M Hassoun
Jul 19, 2011·The American Journal of Pathology·Yu-Mee KimMarlene Rabinovitch
Oct 21, 2011·Journal of Biomechanical Engineering·Katherine D Kavlock, Aaron S Goldstein
Jan 17, 2012·American Journal of Respiratory and Critical Care Medicine·Glenn MarsboomStephen L Archer
Apr 24, 2012·The European Respiratory Journal·Prakash ChelladuraiSoni Savai Pullamsetti
May 15, 2012·American Journal of Physiology. Lung Cellular and Molecular Physiology·Adil AnwarKurt R Stenmark
Sep 8, 2012·American Journal of Respiratory and Critical Care Medicine·Rajkumar SavaiRalph T Schermuly
Oct 23, 2012·American Journal of Physiology. Lung Cellular and Molecular Physiology·Devon ScottWei Tan
Dec 15, 2012·American Journal of Respiratory Cell and Molecular Biology·M Patricia GeorgeKaren A Norris
Jun 25, 2013·Journal of Molecular Medicine : Official Organ of the Gesellschaft Deutscher Naturforscher Und Ärzte·Lin PiaoStephen L Archer
Jul 23, 2013·The Journal of Clinical Investigation·Edda SpiekerkoetterMarlene Rabinovitch

❮ Previous
Next ❯

Citations

Sep 29, 2018·American Journal of Physiology. Heart and Circulatory Physiology·Philip Tannenberg, Karin Tran-Lundmark
Nov 8, 2018·Antioxidants & Redox Signaling·Norah AlruwailiMichael S Wolin
Sep 7, 2019·American Journal of Physiology. Heart and Circulatory Physiology·Thenappan ThenappanE Kenneth Weir
Jul 15, 2020·Pediatric Pulmonology·Eric B McClellanCandice D Fike
Aug 30, 2020·Journal of Cellular and Molecular Medicine·Genfa XiaoLiangdi Xie
Jan 10, 2020·FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology·Chinmayee D BhediKrishna C Penumatsa
May 28, 2020·International Journal of Molecular Sciences·James M MurphySsang-Taek Steve Lim
Apr 9, 2020·International Journal of Molecular Sciences·Jorge NuchePilar Escribano Subías
Aug 21, 2019·Frontiers in Cardiovascular Medicine·Denise BurtenshawPaul A Cahill
Mar 12, 2020·American Journal of Respiratory Cell and Molecular Biology·Katharina JandlGrazyna Kwapiszewska
Jan 8, 2020·Pulmonary Circulation·Mattias ArvidssonGöran Rådegran
Dec 12, 2020·American Journal of Physiology. Heart and Circulatory Physiology·Nikolai Pakhomov, John A Baugh
Oct 1, 2020·American Journal of Physiology. Lung Cellular and Molecular Physiology·Joshua R SheakLaura V Gonzalez Bosc
Dec 18, 2020·Frontiers in Pharmacology·Radhiga ThangaiyanNagarajan Rajendra Prasad
Mar 20, 2021·Frontiers in Cell and Developmental Biology·Han BaoYing-Xin Qi
Feb 11, 2021·American Journal of Physiology. Lung Cellular and Molecular Physiology·Chen-Shan Chen WoodcockStephen Y Chan
Jun 11, 2021·Frontiers in Cell and Developmental Biology·Xiaomin CaiZhipeng Meng
Jun 3, 2021·Journal of Biomechanical Engineering·Eun-Ho Lee, Seungik Baek
May 12, 2021·Journal of Biomechanical Engineering·Caymen NovakSamir Ghadiali
Aug 26, 2021·Journal of Experimental Pharmacology·Ronald Zolty
Aug 25, 2021·Nitric Oxide : Biology and Chemistry·Jiechun Zhu, Guangdong Yang
Jun 16, 2021·American Journal of Respiratory Cell and Molecular Biology·Anjira S AmbadeRachel L Damico
Jan 30, 2021·The European Respiratory Journal·Colin E EvansYou-Yang Zhao
Oct 21, 2021·American Journal of Physiology. Cell Physiology·Ziyi WangJason X-J Yuan
Oct 14, 2021·American Journal of Respiratory and Critical Care Medicine·Elena A GoncharovaSoni S Pullamsetti

❮ Previous
Next ❯

Methods Mentioned

BETA
GTPase

Clinical Trials Mentioned

NCT03428217
NCT02771626
NCT02861300

Related Concepts

Related Feeds

CREs: Gene & Cell Therapy

Gene and cell therapy advances have shown promising outcomes for several diseases. The role of cis-regulatory elements (CREs) is crucial in the design of gene therapy vectors. Here is the latest research on CREs in gene and cell therapy.

© 2022 Meta ULC. All rights reserved