Journal
ANNUAL REVIEW OF PATHOLOGY: MECHANISMS OF DISEASE, VOL 10
Volume 10, Issue -, Pages 371-393Publisher
ANNUAL REVIEWS
DOI: 10.1146/annurev-pathol-012513-104644
Keywords
alveolar proteinosis; interstitial lung disease; respiratory distress syndrome; alveolar capillary dysplasia; pulmonary fibrosis; pulmonary alveolar microlithiasis
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Funding
- NHLBI NIH HHS [HL110964, R01 HL086492, R01 HL095580, HL1093923, U01 HL122642, HL108907, R01 HL103923, U01 HL110964, HL095580, HL122642, R01 HL108907, HL086492] Funding Source: Medline
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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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