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BMC Pulmonary Medicine
Published in: BMC Pulmonary Medicine 1/2014

Open Access 01-12-2014 | Research article

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

Authors: Olaf Eickmeier, Su Youn Kim, Eva Herrmann, Constanze Döring, Ruth Duecker, Sandra Voss, Sibylle Wehner, Christoph Hölscher, Julia Pietzner, Stefan Zielen, Ralf Schubert

Published in: BMC Pulmonary Medicine | Issue 1/2014

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Abstract

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.
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Metadata
Title
Altered mucosal immune response after acute lung injury in a murine model of Ataxia Telangiectasia
Authors
Olaf Eickmeier
Su Youn Kim
Eva Herrmann
Constanze Döring
Ruth Duecker
Sandra Voss
Sibylle Wehner
Christoph Hölscher
Julia Pietzner
Stefan Zielen
Ralf Schubert
Publication date
01-12-2014
Publisher
BioMed Central
Published in
BMC Pulmonary Medicine / Issue 1/2014
Electronic ISSN: 1471-2466
DOI
https://doi.org/10.1186/1471-2466-14-93

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