Alveolar surfactant homeostasis and the pathogenesis of pulmonary disease.
Alveolar surfactant homeostasis and the pathogenesis of pulmonary disease.
复制标题
肺泡表面活性物质动态平衡与肺部疾病发病机制。
DOI：
10.1146/annurev.med.60.041807.123500
复制
发表时间：
2010
影响因子：
10.5
通讯作者：
Weaver TE
中科院分区：
文献类型：
--
作者：
Whitsett JA;Wert SE;Weaver TE
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, SFTPC, 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.
登录
查看更多内容
DOI：
10.1164/rccm.200503-504oc
发表时间：
2005-10-15
影响因子：
24.7
作者：
Bullard, JE;Wert, SE;Nogee, LM
通讯作者：
Nogee, LM
影响因子：
5.1
作者：
Hamvas, A;Nogee, LM;Colten, HR
通讯作者：
Colten, HR
DOI：
10.1073/pnas.101500298
发表时间：
2001-05-22
影响因子：
11.1
作者：
Glasser, SW;Burhans, MS;Whitsett, JA
通讯作者：
Whitsett, JA
DOI：
10.1164/rccm.200804-550oc
发表时间：
2008-10-01
影响因子：
24.7
作者：
Cronkhite, Jennifer T.;Xing, Chao;Garcia, Christine Kim
通讯作者：
Garcia, Christine Kim
适应和增加与错误折叠SP-C的本构表达相关的感染的敏感性。
影响因子：
7.8
作者：
Bridges, James P;Xu, Yan;Na, Cheng-Lun;Wong, Hector R;Weaver, Timothy E
通讯作者：
Weaver, Timothy E