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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MDPI AG
2021
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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) |
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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 |
work_keys_str_mv |
AT inescaldeira developmentalpathwaysunderlyinglungdevelopmentandcongenitallungdisorders AT hugofernandessilva developmentalpathwaysunderlyinglungdevelopmentandcongenitallungdisorders AT danielamachadocosta developmentalpathwaysunderlyinglungdevelopmentandcongenitallungdisorders AT jorgecorreiapinto developmentalpathwaysunderlyinglungdevelopmentandcongenitallungdisorders AT rutesilvamoura developmentalpathwaysunderlyinglungdevelopmentandcongenitallungdisorders |
_version_ |
1718412636436037632 |