Developmental Pathways Underlying Lung Development and Congenital Lung Disorders

Lung organogenesis is a highly coordinated process governed by a network of conserved signaling pathways that ultimately control patterning, growth, and differentiation. This rigorously regulated developmental process culminates with the formation of a fully functional organ. Conversely, failure to...

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Autores principales: Inês Caldeira, Hugo Fernandes-Silva, Daniela Machado-Costa, Jorge Correia-Pinto, Rute Silva Moura
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Publicado: MDPI AG 2021
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spelling oai:doaj.org-article:24fd3238fd69472fa9966c6e00519efd2021-11-25T17:10:06ZDevelopmental Pathways Underlying Lung Development and Congenital Lung Disorders10.3390/cells101129872073-4409https://doaj.org/article/24fd3238fd69472fa9966c6e00519efd2021-11-01T00:00:00Zhttps://www.mdpi.com/2073-4409/10/11/2987https://doaj.org/toc/2073-4409Lung organogenesis is a highly coordinated process governed by a network of conserved signaling pathways that ultimately control patterning, growth, and differentiation. This rigorously regulated developmental process culminates with the formation of a fully functional organ. Conversely, failure to correctly regulate this intricate series of events results in severe abnormalities that may compromise postnatal survival or affect/disrupt lung function through early life and adulthood. Conditions like congenital pulmonary airway malformation, bronchopulmonary sequestration, bronchogenic cysts, and congenital diaphragmatic hernia display unique forms of lung abnormalities. The etiology of these disorders is not yet completely understood; however, specific developmental pathways have already been reported as deregulated. In this sense, this review focuses on the molecular mechanisms that contribute to normal/abnormal lung growth and development and their impact on postnatal survival.Inês CaldeiraHugo Fernandes-SilvaDaniela Machado-CostaJorge Correia-PintoRute Silva MouraMDPI AGarticlecongenital pulmonary airway malformation (CPAM)bronchopulmonary sequestrationbronchogenic cystscongenital diaphragmatic hernia (CDH)congenital malformationsBiology (General)QH301-705.5ENCells, Vol 10, Iss 2987, p 2987 (2021)
institution DOAJ
collection DOAJ
language EN
topic congenital pulmonary airway malformation (CPAM)
bronchopulmonary sequestration
bronchogenic cysts
congenital diaphragmatic hernia (CDH)
congenital malformations
Biology (General)
QH301-705.5
spellingShingle congenital pulmonary airway malformation (CPAM)
bronchopulmonary sequestration
bronchogenic cysts
congenital diaphragmatic hernia (CDH)
congenital malformations
Biology (General)
QH301-705.5
Inês Caldeira
Hugo Fernandes-Silva
Daniela Machado-Costa
Jorge Correia-Pinto
Rute Silva Moura
Developmental Pathways Underlying Lung Development and Congenital Lung Disorders
description Lung organogenesis is a highly coordinated process governed by a network of conserved signaling pathways that ultimately control patterning, growth, and differentiation. This rigorously regulated developmental process culminates with the formation of a fully functional organ. Conversely, failure to correctly regulate this intricate series of events results in severe abnormalities that may compromise postnatal survival or affect/disrupt lung function through early life and adulthood. Conditions like congenital pulmonary airway malformation, bronchopulmonary sequestration, bronchogenic cysts, and congenital diaphragmatic hernia display unique forms of lung abnormalities. The etiology of these disorders is not yet completely understood; however, specific developmental pathways have already been reported as deregulated. In this sense, this review focuses on the molecular mechanisms that contribute to normal/abnormal lung growth and development and their impact on postnatal survival.
format article
author Inês Caldeira
Hugo Fernandes-Silva
Daniela Machado-Costa
Jorge Correia-Pinto
Rute Silva Moura
author_facet Inês Caldeira
Hugo Fernandes-Silva
Daniela Machado-Costa
Jorge Correia-Pinto
Rute Silva Moura
author_sort Inês Caldeira
title Developmental Pathways Underlying Lung Development and Congenital Lung Disorders
title_short Developmental Pathways Underlying Lung Development and Congenital Lung Disorders
title_full Developmental Pathways Underlying Lung Development and Congenital Lung Disorders
title_fullStr Developmental Pathways Underlying Lung Development and Congenital Lung Disorders
title_full_unstemmed Developmental Pathways Underlying Lung Development and Congenital Lung Disorders
title_sort developmental pathways underlying lung development and congenital lung disorders
publisher MDPI AG
publishDate 2021
url https://doaj.org/article/24fd3238fd69472fa9966c6e00519efd
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AT danielamachadocosta developmentalpathwaysunderlyinglungdevelopmentandcongenitallungdisorders
AT jorgecorreiapinto developmentalpathwaysunderlyinglungdevelopmentandcongenitallungdisorders
AT rutesilvamoura developmentalpathwaysunderlyinglungdevelopmentandcongenitallungdisorders
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