Endothelial dysfunction in pulmonary hypertension:Cause or consequence?
Autor: | J. Wouter Jukema, Kondababu Kurakula, Marie-José Goumans, Valérie F E D Smolders, Paul H.A. Quax, Olga Tura-Ceide |
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Jazyk: | angličtina |
Rok vydání: | 2021 |
Předmět: |
0301 basic medicine
TGF-β medicine.medical_specialty Endothelium Medicine (miscellaneous) Inflammation Review 030204 cardiovascular system & hematology General Biochemistry Genetics and Molecular Biology endothelial dysfunction Vascular remodelling in the embryo TGF-βepigenetics 03 medical and health sciences vasoactive factors 0302 clinical medicine medicine.artery Internal medicine pulmonary hypertension EndoMT medicine Thrombus Endothelial dysfunction lcsh:QH301-705.5 epigenetics business.industry medicine.disease Pulmonary hypertension Endothelial stem cell 030104 developmental biology medicine.anatomical_structure lcsh:Biology (General) inflammation Pulmonary artery Cardiology medicine.symptom business |
Zdroj: | Kurakula, K, Smolders, V F E D, Tura-Ceide, O, Wouter Jukema, J, Quax, P H A & Goumans, M J 2021, ' Endothelial dysfunction in pulmonary hypertension : Cause or consequence? ', Biomedicines, vol. 9, no. 1, 57, pp. 1-23 . https://doi.org/10.3390/biomedicines9010057 Biomedicines, 9(1). MDPI Biomedicines, Vol 9, Iss 57, p 57 (2021) Biomedicines |
Popis: | Pulmonary arterial hypertension (PAH) is a rare, complex, and progressive disease that is characterized by the abnormal remodeling of the pulmonary arteries that leads to right ventricular failure and death. Although our understanding of the causes for abnormal vascular remodeling in PAH is limited, accumulating evidence indicates that endothelial cell (EC) dysfunction is one of the first triggers initiating this process. EC dysfunction leads to the activation of several cellular signalling pathways in the endothelium, resulting in the uncontrolled proliferation of ECs, pulmonary artery smooth muscle cells, and fibroblasts, and eventually leads to vascular remodelling and the occlusion of the pulmonary blood vessels. Other factors that are related to EC dysfunction in PAH are an increase in endothelial to mesenchymal transition, inflammation, apoptosis, and thrombus formation. In this review, we outline the latest advances on the role of EC dysfunction in PAH and other forms of pulmonary hypertension. We also elaborate on the molecular signals that orchestrate EC dysfunction in PAH. Understanding the role and mechanisms of EC dysfunction will unravel the therapeutic potential of targeting this process in PAH. |
Databáze: | OpenAIRE |
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