Altered mucosal immune response after acute lung injury in a murine model of Ataxia Telangiectasia

被引：18

作者：

Eickmeier, Olaf ^{[1
]}

Kim, Su Youn ^{[1
]}

Herrmann, Eva ^{[2
]}

Doering, Constanze ^{[3
]}

Duecker, Ruth ^{[1
]}

Voss, Sandra ^{[1
]}

Wehner, Sibylle ^{[1
]}

Hoelscher, Christoph ^{[4
]}

Pietzner, Julia ^{[1
]}

Zielen, Stefan ^{[1
]}

Schubert, Ralf ^{[1
]}

机构：

[1] Goethe Univ Frankfurt, D-60590 Frankfurt, Germany

[2] Goethe Univ Frankfurt, Inst Biostat & Math Modeling, D-60590 Frankfurt, Germany

[3] Goethe Univ Frankfurt, Senckenberg Inst Pathol, D-60590 Frankfurt, Germany

[4] Res Ctr Borstel, Div Infect Immunol, Borstel, Germany

来源：

BMC PULMONARY MEDICINE | 2014年 / 14卷

关键词：

Ataxia telangiectasia; ATM; Acute lung injury; Inflammation; ATM-DEFICIENT MICE; DISEASE; ASPIRATION; RECOMBINATION; PATHOGENESIS; NEUTROPHILS; INFECTIONS; RESOLUTION; PARADIGM; PREVENTS;

D O I：

10.1186/1471-2466-14-93

中图分类号：

R56 [呼吸系及胸部疾病];

学科分类号：

摘要：

Background: Ataxia telangiectasia (A-T) is a rare but devastating and progressive disorder characterized by cerebellar dysfunction, lymphoreticular malignancies and recurrent sinopulmonary infections. In A-T, disease of the respiratory system causes significant morbidity and is a frequent cause of death. Methods: We used a self-limited murine model of hydrochloric acid-induced acute lung injury (ALI) to determine the inflammatory answer due to mucosal injury in Atm (A-T mutated)-deficient mice (Atm(-/-)). Results: ATM deficiency increased peak lung inflammation as demonstrated by bronchoalveolar lavage fluid (BALF) neutrophils and lymphocytes and increased levels of BALF pro-inflammatory cytokines (e. g. IL-6, TNF). Furthermore, bronchial epithelial damage after ALI was increased in Atm(-/-) mice. ATM deficiency increased airway resistance and tissue compliance before ALI was performed. Conclusions: Together, these findings indicate that ATM plays a key role in inflammatory response after airway mucosal injury.

引用

页数：9

共 41 条

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