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

  • 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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Author:Olaf EickmeierORCiDGND, Su Youn Kim, Eva HerrmannORCiDGND, Constanze Döring, Ruth Pia DückerORCiDGND, Sandra Voss, Sibylle Wehner, Christoph Hölscher, Julia Pietzner, Stefan ZielenORCiDGND, Ralf SchubertORCiDGND
URN:urn:nbn:de:hebis:30:3-349851
DOI:https://doi.org/10.1186/1471-2466-14-93
ISSN:1471-2466
Pubmed Id:https://pubmed.ncbi.nlm.nih.gov/24884546
Parent Title (English):BMC pulmonary medicine
Publisher:BioMed Central
Place of publication:London
Document Type:Article
Language:English
Date of Publication (online):2014/05/29
Date of first Publication:2014/05/29
Publishing Institution:Universitätsbibliothek Johann Christian Senckenberg
Release Date:2014/10/29
Tag:ATM; acute lung injury; ataxia telangiectasia; inflammation
Volume:14
Issue:93
Page Number:9
Note:
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.
HeBIS-PPN:366630210
Institutes:Medizin / Medizin
Dewey Decimal Classification:6 Technik, Medizin, angewandte Wissenschaften / 61 Medizin und Gesundheit / 610 Medizin und Gesundheit
Sammlungen:Universitätspublikationen
Licence (German):License LogoCreative Commons - Namensnennung 2.0