title: Altered mucosal immune response after acute lung injury in a murine model of Ataxia Telangiectasia.
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.
authors.affiliation: Pediatric Pulmonology, Allergy and Cystic Fibrosis, Johann Wolfgang Goethe- University, Theodor-Stern-Kai 7, Frankfurt D-60590, Germany. olaf.eickmeier@kgu.de.
authors.forename: Olaf
authors.initials: O
authors.lastname: Eickmeier
authors.forename: Su Youn
authors.initials: SY
authors.lastname: Kim
authors.forename: Eva
authors.initials: E
authors.lastname: Herrmann
authors.forename: Constanze
authors.initials: C
authors.lastname: Döring
authors.forename: Ruth
authors.initials: R
authors.lastname: Duecker
authors.forename: Sandra
authors.initials: S
authors.lastname: Voss
authors.forename: Sibylle
authors.initials: S
authors.lastname: Wehner
authors.forename: Christoph
authors.initials: C
authors.lastname: Hölscher
authors.forename: Julia
authors.initials: J
authors.lastname: Pietzner
authors.forename: Stefan
authors.initials: S
authors.lastname: Zielen
authors.forename: Ralf
authors.initials: R
authors.lastname: Schubert
mesh_terms: D055371:Acute Lung Injury / Q000276:immunology* / Q000473:pathology*; D000818:Animals; D001260:Ataxia Telangiectasia / Q000276:immunology* / Q000473:pathology; D064007:Ataxia Telangiectasia Mutated Proteins / Q000172:deficiency; D001707:Biopsy, Needle; D016207:Cytokines / Q000276:immunology; D004195:Disease Models, Animal; D018928:Immunity, Mucosal / Q000502:physiology*; D007150:Immunohistochemistry; D008297:Male; D051379:Mice; D008815:Mice, Inbred Strains; D009504:Neutrophils / Q000276:immunology; D011897:Random Allocation; D012016:Reference Values; D014409:Tumor Necrosis Factor-alpha / Q000276:immunology
chemical_list: D016207:Cytokines; D014409:Tumor Necrosis Factor-alpha; D064007:Ataxia Telangiectasia Mutated Proteins; C576325:Atm protein, mouse