TY  - JOUR
A1  - Eickmeier, Olaf
A1  - Kim, Su Youn
A1  - Herrmann, Eva
A1  - Döring, Constanze
A1  - Dücker, Ruth Pia
A1  - Voss, Sandra
A1  - Wehner, Sibylle
A1  - Hölscher, Christoph
A1  - Pietzner, Julia
A1  - Zielen, Stefan
A1  - Schubert, Ralf
T1  - Altered mucosal immune response after acute lung injury in a murine model of Ataxia Telangiectasia
T2  - BMC pulmonary medicine
N2  - 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.
KW  - ataxia telangiectasia
KW  - ATM
KW  - acute lung injury
KW  - inflammation
Y1  - 2014
UR  - http://publikationen.ub.uni-frankfurt.de/frontdoor/index/index/docId/34985
UR  - https://nbn-resolving.org/urn:nbn:de:hebis:30:3-349851
SN  - 1471-2466
N1  - Copyright © 2014 Eickmeier et al.; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited.
VL  - 14
IS  - 93
PB  - BioMed Central
CY  - London
ER  -