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

作者：Eickmeier Olaf^*; Kim Su Youn; Herrmann Eva; Doering Constanze; Duecker Ruth; Voss Sandra; Wehner Sibylle; Hoelscher Christoph; Pietzner Julia; Zielen Stefan; Schubert Ralf

来源：BMC Pulmonary Medicine, 2014, 14(1): 93.

DOI：10.1186/1471-2466-14-93

• Ataxia telangiectasia
• ATM
• Acute lung injury
• Inflammation

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. %26lt;br%26gt;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(-/-)). %26lt;br%26gt;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. %26lt;br%26gt;Conclusions: Together, these findings indicate that ATM plays a key role in inflammatory response after airway mucosal injury.

• 出版日期2014-5-29