Alveolar injury and regeneration following deletion of ABCA3.
Alveolar injury and regeneration following deletion of ABCA3.
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ABCA3 缺失后的肺泡损伤和再生。
DOI：
10.1172/jci.insight.97381
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发表时间：
2017
期刊：
影响因子：
8
通讯作者：
Whitsett,JeffreyA
中科院分区：
文献类型：
--
作者：
Rindler,TaraN;Stockman,CourtneyA;Filuta,AlyssaL;Brown,KariM;Snowball,JohnM;Zhou,Wenjia;Veldhuizen,Ruud;Zink,ErikaM;Dautel,SydneyE;Clair,Geremy;Ansong,Charles;Xu,Yan;Bridges,JamesP;Whitsett,JeffreyA
Adaptation to air breathing after birth is dependent upon the synthesis and secretion of pulmonary surfactant by alveolar type 2 (AT2) cells. Surfactant, a complex mixture of phospholipids and proteins, is secreted into the alveolus, where it reduces collapsing forces at the air-liquid interface to maintain lung volumes during the ventilatory cycle. ABCA3, an ATP-dependent Walker domain containing transport protein, is required for surfactant synthesis and lung function at birth. Mutations in ABCA3 cause severe surfactant deficiency and respiratory failure in newborn infants. We conditionally deleted the Abca3 gene in AT2 cells in the mature mouse lung. Loss of ABCA3 caused alveolar cell injury and respiratory failure. ABCA3-related lung dysfunction was associated with surfactant deficiency, inflammation, and alveolar-capillary leak. Extensive but incomplete deletion of ABCA3 caused alveolar injury and inflammation, and it initiated proliferation of progenitor cells, restoring ABCA3 expression, lung structure, and function. M2-like macrophages were recruited to sites of AT2 cell proliferation during the regenerative process and were present in lung tissue from patients with severe lung disease caused by mutations in ABCA3. The remarkable and selective regeneration of ABCA3-sufficient AT2 progenitor cells provides plausible approaches for future correction of ABCA3 and other genetic disorders associated with surfactant deficiency and acute interstitial lung disease.
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影响因子：
23.9
作者：
Lechner AJ;Driver IH;Lee J;Conroy CM;Nagle A;Locksley RM;Rock JR
通讯作者：
Rock JR
DOI：
10.1164/ajrccm.153.1.8542113
发表时间：
1996
影响因子：
24.7
作者：
A. Günther;C. Siebert;R. Schmidt;S. Ziegler;F. Grimminger;M. Yabut;B. Temmesfeld;D. Walmrath;H. Morr;W. Seeger
通讯作者：
A. Günther;C. Siebert;R. Schmidt;S. Ziegler;F. Grimminger;M. Yabut;B. Temmesfeld;D. Walmrath;H. Morr;W. Seeger
DOI：
10.1016/j.bbrc.2007.05.219
发表时间：
2007-08-10
影响因子：
3.1
作者：
Hammel, Markus;Michel, Geert;Holzinger, Andreas
通讯作者：
Holzinger, Andreas
影响因子：
25
作者：
Madisen L;Zwingman TA;Sunkin SM;Oh SW;Zariwala HA;Gu H;Ng LL;Palmiter RD;Hawrylycz MJ;Jones AR;Lein ES;Zeng H
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Zeng H
约束固着滴作为测量肺表面活性剂系统中低表面张力的新配置。
影响因子：
3.3
作者：
L. M. Yu;James J. Lu;Yawen W. Chan;A. Ng;Ling Zhang;M. Hoorfar;Z. Policova;K. Grundke;A. Neumann
通讯作者：
A. Neumann