Altered stability of pulmonary surfactant in SP-C-deficient mice
Altered stability of pulmonary surfactant in SP-C-deficient mice
复制标题
DOI：
10.1073/pnas.101500298
复制
发表时间：
2001-05-22
影响因子：
11.1
通讯作者：
Whitsett, JA
中科院分区：
文献类型：
--
作者：
Glasser, SW;Burhans, MS;Whitsett, JA
表面活性剂蛋白 C (SP-C) 基因编码一种由肺泡上皮细胞产生的极其疏水的 4-kDa 肽。为了了解 SP-C 在肺功能中的作用，制备了 SP-C 缺陷 (-/-) 小鼠。 SP-C(-/-)小鼠出生时存活，正常生长至成年期，无明显肺部异常，SP-C(-/-)小鼠肺部未检测到SP-C mRNA，SP-C(-/-)小鼠肺泡灌洗液Western blot检测也未检测到成熟的SP-C蛋白。肺泡损伤中其他表面活性蛋白（A、B、D）的水平与野生型小鼠相当。 SP-C (-/-) 和 SP-C (+/+) 小鼠的表面活性剂库大小、表面活性剂合成和肺形态相似。 SP-C (-/-) II 型细胞中存在板层体，肺泡腔中存在管状髓磷脂。肺力学研究表明，在低呼气末正压时，肺迟滞性（用于反映能量耗散力和组织弹性特性之间的机械耦合的术语）异常。 SP-C (-/-) 小鼠的表面活性剂捕获气泡的稳定性显着降低，表明 SP-C 在低肺容量下稳定表面活性剂方面发挥着作用，这种情况可能伴随婴儿和成人呼吸窘迫综合征。
The surfactant protein C (SP-C) gene encodes an extremely hydrophobic, 4-kDa peptide produced by alveolar epithelial cells in the lung. To discern the role of SP-C in lung function, SP-C-deficient (-/-) mice were produced. The SP-C (-/-) mice were viable at birth and grew normally to adulthood without apparent pulmonary abnormalities, SP-C mRNA was not detected in the lungs of SP-C(-/-) mice, nor was mature SP-C protein detected by Western blot of alveolar lavage from SP-C (-/-) mice. The levels of the other surfactant proteins (A, B, D) in alveolar ravage were comparable to those in wild-type mice. Surfactant pool sizes, surfactant synthesis, and lung morphology were similar in SP-C (-/-) and SP-C (+/+) mice. Lamellar bodies were present in SP-C (-/-) type II cells, and tubular myelin was present in the alveolar lumen. Lung mechanics studies demonstrated abnormalities in lung hysteresivity (a term used to reflect the mechanical coupling between energy dissipative forces and tissue-elastic properties) at low, positive-end, expiratory pressures. The stability of captive bubbles with surfactant from the SP-C (-/-) mice was decreased significantly, indicating that SP-C plays a role in the stabilization of surfactant at low lung volumes, a condition that may accompany respiratory distress syndrome in infants and adults.