BMP type II receptor as a therapeutic target in pulmonary arterial hypertension = BMPR2 como diana terapeutica en la hipertensión arterial pulmon

Pulmonary arterial hypertension (PAH) is a chronic disease characterized by a progressive elevation in mean pulmonary arterial pressure. This occurs due to abnormal remodeling of small peripheral lung vasculature resulting in progressive occlusion of the artery lumen that eventually causes right hea...

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Autor Principal: Gómez Puerto, María Catalina; Orriols, Mar; Ten Dijke, Peter
Formato: Artículo (Article)
Lenguaje:Desconocido (Unknown)
Publicado: Cellular and Molecular Life Sciences; Vol. 74, No. 16 2017
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Acceso en línea:http://babel.banrepcultural.org/cdm/ref/collection/p17054coll23/id/814
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spelling ir-p17054coll23-8142020-09-29 BMP type II receptor as a therapeutic target in pulmonary arterial hypertension = BMPR2 como diana terapeutica en la hipertensión arterial pulmon Gómez Puerto, María Catalina; Orriols, Mar; Ten Dijke, Peter Pulmonary arterial hypertension (PAH) is a chronic disease characterized by a progressive elevation in mean pulmonary arterial pressure. This occurs due to abnormal remodeling of small peripheral lung vasculature resulting in progressive occlusion of the artery lumen that eventually causes right heart failure and death. The most common cause of PAH is inactivating mutations in the gene encoding a bone morphogenetic protein type II receptor (BMPRII). Current therapeutic options for PAH are limited and focused mainly on reversal of pulmonary vasoconstriction and proliferation of vascular cells. Although these treatments can relieve disease symptoms, PAH remains a progressive lethal disease. Emerging data suggest that restoration of BMPRII signaling in PAH is a promising alternative that could prevent and reverse pulmonary vascular remodeling. Here we will focus on recent advances in rescuing BMPRII expression, function or signaling to prevent and reverse pulmonary vascular remodeling in PAH and its feasibility for clinical translation. Furthermore, we summarize the role of described miRNAs that directly target the BMPR2 gene in blood vessels. We discuss the therapeutic potential and the limitations of promising new approaches to restore BMPRII signaling in PAH patients. Different mutations in BMPR2 and environmental/genetic factors make PAH a heterogeneous disease and it is thus likely that the best approach will be patient-tailored therapies. Autofagia; Células endoteliales; Células musculares lisas vasculares; Cell; Endothelial cell; Inflamación; Inflammation; Remodelación vascular; Remodeling and autophagy; Signal transduction; Vascular smooth muscle; Vascular Tecnología; Tecnología / Ciencias médicas Medicina; Tecnología / Ciencias médicas Medicina / Enfermedades 2017 Cellular and Molecular Life Sciences; Vol. 74, No. 16 PDF Artículo ENG - Inglés Colfuturo © Derechos reservados del autor http://babel.banrepcultural.org/cdm/ref/collection/p17054coll23/id/814
institution Biblioteca Virtual Banco de la República - Colecciones digitales
collection Custom
language Desconocido (Unknown)
topic Autofagia; Células endoteliales; Células musculares lisas vasculares; Cell; Endothelial cell; Inflamación; Inflammation; Remodelación vascular; Remodeling and autophagy; Signal transduction; Vascular smooth muscle; Vascular
Tecnología; Tecnología / Ciencias médicas Medicina; Tecnología / Ciencias médicas Medicina / Enfermedades
spellingShingle Autofagia; Células endoteliales; Células musculares lisas vasculares; Cell; Endothelial cell; Inflamación; Inflammation; Remodelación vascular; Remodeling and autophagy; Signal transduction; Vascular smooth muscle; Vascular
Tecnología; Tecnología / Ciencias médicas Medicina; Tecnología / Ciencias médicas Medicina / Enfermedades
Gómez Puerto, María Catalina; Orriols, Mar; Ten Dijke, Peter
BMP type II receptor as a therapeutic target in pulmonary arterial hypertension = BMPR2 como diana terapeutica en la hipertensión arterial pulmon
description Pulmonary arterial hypertension (PAH) is a chronic disease characterized by a progressive elevation in mean pulmonary arterial pressure. This occurs due to abnormal remodeling of small peripheral lung vasculature resulting in progressive occlusion of the artery lumen that eventually causes right heart failure and death. The most common cause of PAH is inactivating mutations in the gene encoding a bone morphogenetic protein type II receptor (BMPRII). Current therapeutic options for PAH are limited and focused mainly on reversal of pulmonary vasoconstriction and proliferation of vascular cells. Although these treatments can relieve disease symptoms, PAH remains a progressive lethal disease. Emerging data suggest that restoration of BMPRII signaling in PAH is a promising alternative that could prevent and reverse pulmonary vascular remodeling. Here we will focus on recent advances in rescuing BMPRII expression, function or signaling to prevent and reverse pulmonary vascular remodeling in PAH and its feasibility for clinical translation. Furthermore, we summarize the role of described miRNAs that directly target the BMPR2 gene in blood vessels. We discuss the therapeutic potential and the limitations of promising new approaches to restore BMPRII signaling in PAH patients. Different mutations in BMPR2 and environmental/genetic factors make PAH a heterogeneous disease and it is thus likely that the best approach will be patient-tailored therapies.
format Artículo (Article)
author Gómez Puerto, María Catalina; Orriols, Mar; Ten Dijke, Peter
author_facet Gómez Puerto, María Catalina; Orriols, Mar; Ten Dijke, Peter
author_sort Gómez Puerto, María Catalina; Orriols, Mar; Ten Dijke, Peter
title BMP type II receptor as a therapeutic target in pulmonary arterial hypertension = BMPR2 como diana terapeutica en la hipertensión arterial pulmon
title_short BMP type II receptor as a therapeutic target in pulmonary arterial hypertension = BMPR2 como diana terapeutica en la hipertensión arterial pulmon
title_full BMP type II receptor as a therapeutic target in pulmonary arterial hypertension = BMPR2 como diana terapeutica en la hipertensión arterial pulmon
title_fullStr BMP type II receptor as a therapeutic target in pulmonary arterial hypertension = BMPR2 como diana terapeutica en la hipertensión arterial pulmon
title_full_unstemmed BMP type II receptor as a therapeutic target in pulmonary arterial hypertension = BMPR2 como diana terapeutica en la hipertensión arterial pulmon
title_sort bmp type ii receptor as a therapeutic target in pulmonary arterial hypertension = bmpr2 como diana terapeutica en la hipertensión arterial pulmon
publisher Cellular and Molecular Life Sciences; Vol. 74, No. 16
publishDate 2017
url http://babel.banrepcultural.org/cdm/ref/collection/p17054coll23/id/814
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score 11,828437