Alveolar Surfactant Homeostasis and the Pathogenesis of Pulmonary Disease

被引:309
作者
Whitsett, Jeffrey A. [1 ]
Wert, Susan E.
Weaver, Timothy E.
机构
[1] Cincinnati Childrens Hosp, Med Ctr, Perinatal Inst, Sect Neonatol Perinatal & Pulm Biol, Cincinnati, OH 45229 USA
来源
ANNUAL REVIEW OF MEDICINE | 2010年 / 61卷
关键词
alveolar proteinosis; interstitial lung disease; respiratory distress syndrome; pulmonary fibrosis; pulmonary alveolar microlithiasis; COLONY-STIMULATING FACTOR; ENDOPLASMIC-RETICULUM STRESS; INTERSTITIAL LUNG-DISEASE; PROTEIN-C; ABCA3; MUTATIONS; DEFICIENT MICE; SP-B; GENE; EXPRESSION; MICROLITHIASIS;
D O I
10.1146/annurev.med.60.041807.123500
中图分类号
R-3 [医学研究方法]; R3 [基础医学];
学科分类号
1001 ;
摘要
The alveolar region of the lung creates an extensive epithelial surface that mediates the transfer of oxygen and carbon dioxide required for respiration after birth. Maintenance of pulmonary function depends on the function of type II epithelial cells that synthesize and secrete pulmonary surfactant lipids and proteins, reducing the collapsing forces created at the air-liquid interface in the alveoli. Genetic and acquired disorders associated with the surfactant system cause both acute and chronic lung disease. Mutations in the ABCA3, SFTPA, SFTPB, SPTPC, SCL34A2, and TERT genes disrupt type II cell function and/or surfactant homeostasis, causing neonatal respiratory failure and chronic interstitial lung disease. Defects in GM-CSF receptor function disrupt surfactant clearance, causing pulmonary alveolar proteinosis. Abnormalities in the surfactant system and disruption of type II cell homeostasis underlie the pathogenesis of pulmonary disorders previously considered idiopathic, providing the basis for improved diagnosis and therapies of these rare lung diseases.
引用
收藏
页码:105 / 119
页数:15
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