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Updated: Apr 18, 2026

Surfactant Depletion Combined with Injurious Ventilation Results in a Reproducible Model of the Acute Respiratory Distress Syndrome ARDS
Published on: April 7, 2021
Diseases of pulmonary surfactant homeostasis
Jeffrey A Whitsett1, Susan E Wert, Timothy E Weaver
1Divisions of Neonatology, Perinatal Biology, and Pulmonary Biology, Perinatal Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio 45229; email: jeff.whitsett@cchmc.org , susan.wert@cchmc.org , tim.weaver@cchmc.org.
Insights
Pulmonary surfactant, crucial for lung function, is key in treating respiratory distress syndrome in preterm infants. Genetic defects in surfactant homeostasis cause severe lung diseases in infants.
Area of Science:
- Pulmonary physiology and biochemistry
- Molecular and cellular biology
Background:
- Pulmonary surfactant is vital for lung function, especially in preterm infants with respiratory distress syndrome.
- Advances have identified key components like surfactant proteins and regulatory networks.
Purpose of the Study:
- To review the pulmonary surfactant system.
- To explore genetic causes of lung disease linked to disrupted alveolar homeostasis.
Main Methods:
- Review of physiological and biochemical studies.
- Analysis of genetic mutations affecting surfactant homeostasis.
- Insights from biophysical and transgenic mouse models.
Main Results:
- Understanding of molecular processes regulating surfactant production and function.
- Association of gene mutations with severe neonatal lung disease.
- Elucidation of mechanisms in surfactant protein and alveolar homeostasis.
Conclusions:
- The pulmonary surfactant system is critical for lung health.
- Genetic disruptions in surfactant homeostasis lead to significant lung pathologies.
- This knowledge aids in understanding and potentially treating diverse pulmonary disorders.
Abstract:
Advances in physiology and biochemistry have provided fundamental insights into the role of pulmonary surfactant in the pathogenesis and treatment of preterm infants with respiratory distress syndrome. Identification of the surfactant proteins, lipid transporters, and transcriptional networks regulating their expression has provided the tools and insights needed to discern the molecular and cellular processes regulating the production and function of pulmonary surfactant prior to and after birth. Mutations in genes regulating surfactant homeostasis have been associated with severe lung disease in neonates and older infants. Biophysical and transgenic mouse models have provided insight into the mechanisms underlying surfactant protein and alveolar homeostasis. These studies have provided the framework for understanding the structure and function of pulmonary surfactant, which has informed understanding of the pathogenesis of diverse pulmonary disorders previously considered idiopathic. This review considers the pulmonary surfactant system and the genetic causes of acute and chronic lung disease caused by disruption of alveolar homeostasis.
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