Research: Developmental Pathways Underlying Lung Development and Congenital Lung Disorders


. 2021 Nov 2;10(11):2987. doi: 10.3390/cells10112987.

Developmental Pathways Underlying Lung Development and Congenital Lung Disorders

Inês Caldeira 1 2Hugo Fernandes-Silva 1 2 3Daniela Machado-Costa 1 2Jorge Correia-Pinto 1 2 4Rute Silva Moura 1 2Affiliations expand

Free PMC article


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.

Keywords: bronchogenic cysts; bronchopulmonary sequestration; congenital diaphragmatic hernia (CDH); congenital malformations; congenital pulmonary airway malformation (CPAM).

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