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Open Access Research article

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

Olaf Eickmeier1*, Su Youn Kim1, Eva Herrmann2, Constanze Döring3, Ruth Duecker1, Sandra Voss1, Sibylle Wehner4, Christoph Hölscher5, Julia Pietzner1, Stefan Zielen1 and Ralf Schubert1

Author Affiliations

1 Pediatric Pulmonology, Allergy and Cystic Fibrosis, Johann Wolfgang Goethe- University, Theodor-Stern-Kai 7, Frankfurt D-60590, Germany

2 Institute of Biostatistics and Mathematical Modeling, Johann Wolfgang Goethe-University, Frankfurt, Germany

3 Senckenberg Institute of Pathology, Johann Wolfgang Goethe- University, Frankfurt, Germany

4 Pediatric Hematology and Oncology, Johann Wolfgang Goethe- University, Frankfurt, Germany

5 Division of Infection Immunology, Research Center Borstel, Borstel, Germany

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BMC Pulmonary Medicine 2014, 14:93  doi:10.1186/1471-2466-14-93

Published: 29 May 2014



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.


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-/-).


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.


Together, these findings indicate that ATM plays a key role in inflammatory response after airway mucosal injury.

Ataxia telangiectasia; ATM; Acute lung injury; Inflammation