Surfactant protein C: Its unique properties and emerging immunomodulatory role in the lung

被引:97
作者
Mulugeta, Surafel [1 ]
Beers, Michael F. [1 ]
机构
[1] Univ Penn, Sch Med, Dept Med, Div Pulm & Crit Care, Philadelphia, PA 19104 USA
关键词
interstitial lung disease; respiratory infection; pulmonary immunity; SP-C mutation; conformational disease; surfactant;
D O I
10.1016/j.micinf.2006.04.009
中图分类号
R392 [医学免疫学]; Q939.91 [免疫学];
学科分类号
100102 ;
摘要
Surfactant protein C (SP-C) is a highly hydrophobic protein found in pulmonary surfactant. SP-C is synthesized exclusively in alveolar type II cells as a 21 kDa integral membrane precursor protein and subsequently proteolytically processed to a 3.7 kDa secretory protein. SP-C enhances the adsorption and spreading of phospholipids at the air-liquid interface thereby promoting the surface tension-lowering properties of surfactant. The importance of SP-C in normal lung function is underscored by the recent findings of inflammatory lung diseases associated both with absence of alveolar SP-C and with cellular expression of mutant SP-C isoforms. This review examines our current understanding of the role of SP-C in maintaining alveolar epithelial homeostasis and the potential role of abnormal SP-C expression in the development of lung diseases with particular emphasis on microbial pulmonary infection and inflammation. (c) 2006 Published by Elsevier SAS.
引用
收藏
页码:2317 / 2323
页数:7
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