Diseases of pulmonary surfactant homeostasis

JA Whitsett, SE Wert, TE Weaver - Annual Review of Pathology …, 2015 - annualreviews.org
JA Whitsett, SE Wert, TE Weaver
Annual Review of Pathology: Mechanisms of Disease, 2015annualreviews.org
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 …
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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