Endothelial Dysfunction and Disruption in Pulmonary Hypertension

A number of systemic diseases lead to pulmonary hypertension (PH), a serious disorder with a high morbidity and mortality rate. Irrespective of the underlying disease, endothelial dysfunction or disruption plays a key role in the initiation and progression of PH. Endothelial dysfunction and disrupti...

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Autor principal: Mathew, Rajamma
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Publicado: IntechOpen 2020
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Acceso en línea:https://www.intechopen.com/chapters/71921
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author Mathew, Rajamma
author_browse Mathew, Rajamma
author_facet Mathew, Rajamma
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description A number of systemic diseases lead to pulmonary hypertension (PH), a serious disorder with a high morbidity and mortality rate. Irrespective of the underlying disease, endothelial dysfunction or disruption plays a key role in the initiation and progression of PH. Endothelial dysfunction and disruption result in impaired vascular relaxation response, activation of proliferative pathways leading to medial hypertrophy and PH. Endothelial cells (EC) play a crucial role in regulating vascular tone and maintaining homeostasis. Caveolin-1, a 21-22 kD membrane protein, interacts with a number of transducing factors and maintains them in a negative conformation. Disruption of EC results in endothelial caveolin-1 loss and reciprocal activation of proliferative pathways leading to PH, and the accompanying loss of PECAM1 and vascular endothelial cadherin results in barrier dysfunction. These changes lead to the irreversibility of PH. Hypoxia-induced PH is not accompanied by endothelial disruption or caveolin-1 loss but is associated with caveolin-1 dysfunction and the activation of proliferative pathways. Removal of hypoxic exposure results in the reversal of the disease. Thus, EC integrity is an important factor that determines irreversibility vs. reversibility of PH. This chapter will discuss normal EC function and the differences encountered in PH following EC disruption and EC dysfunction.
doi_str_mv 10.5772/intechopen.92177
first_indexed 2026-10-15T21:38:23Z
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spelling intech-719212020-05-04 Endothelial Dysfunction and Disruption in Pulmonary Hypertension Rajamma Mathew Health Sciences A number of systemic diseases lead to pulmonary hypertension (PH), a serious disorder with a high morbidity and mortality rate. Irrespective of the underlying disease, endothelial dysfunction or disruption plays a key role in the initiation and progression of PH. Endothelial dysfunction and disruption result in impaired vascular relaxation response, activation of proliferative pathways leading to medial hypertrophy and PH. Endothelial cells (EC) play a crucial role in regulating vascular tone and maintaining homeostasis. Caveolin-1, a 21-22 kD membrane protein, interacts with a number of transducing factors and maintains them in a negative conformation. Disruption of EC results in endothelial caveolin-1 loss and reciprocal activation of proliferative pathways leading to PH, and the accompanying loss of PECAM1 and vascular endothelial cadherin results in barrier dysfunction. These changes lead to the irreversibility of PH. Hypoxia-induced PH is not accompanied by endothelial disruption or caveolin-1 loss but is associated with caveolin-1 dysfunction and the activation of proliferative pathways. Removal of hypoxic exposure results in the reversal of the disease. Thus, EC integrity is an important factor that determines irreversibility vs. reversibility of PH. This chapter will discuss normal EC function and the differences encountered in PH following EC disruption and EC dysfunction. IntechOpen 2020-05-04 Chapter, Part Of Book https://www.intechopen.com/chapters/71921 doi:10.5772/intechopen.92177 en ISBN:978-1-83881-949-1 https://creativecommons.org/licenses/by/3.0/ https://www.intechopen.com/books/7016 ; Cardiovascular Risk Factors in Pathology
spellingShingle Health Sciences
Mathew, Rajamma
Endothelial Dysfunction and Disruption in Pulmonary Hypertension
title Endothelial Dysfunction and Disruption in Pulmonary Hypertension
title_full Endothelial Dysfunction and Disruption in Pulmonary Hypertension
title_fullStr Endothelial Dysfunction and Disruption in Pulmonary Hypertension
title_full_unstemmed Endothelial Dysfunction and Disruption in Pulmonary Hypertension
title_short Endothelial Dysfunction and Disruption in Pulmonary Hypertension
title_sort endothelial dysfunction and disruption in pulmonary hypertension
topic Health Sciences
url https://www.intechopen.com/chapters/71921
work_keys_str_mv AT mathewrajamma endothelialdysfunctionanddisruptioninpulmonaryhypertension