PT  - JOURNAL ARTICLE
AU  - Tobias Müller
AU  - Sanja Cicko
AU  - Marco Idzko
TI  - CD39 deficiency results in increased inflammation in an animal model of pulmonary fibrosis
DP  - 2014 Sep 01
TA  - European Respiratory Journal
PG  - P3908
VI  - 44
IP  - Suppl 58
4099  - http://erj.ersjournals.com/content/44/Suppl_58/P3908.short
4100  - http://erj.ersjournals.com/content/44/Suppl_58/P3908.full
SO  - Eur Respir J2014 Sep 01; 44
AB  - Idiopathic pulmonary fibrosis is a progressive disease with a poor prognosis and very few therapeutic options available at the moment. Extracellular nucleotides such as ATP have gained attention as important regulators in both inflammation and tissue remodelling. The levels of extracellular nucleotides are tightly regulated by the molecule CD39, a membrane bound ectonucleotidase. In this study we investigated the role of CD39 in the context of bleomycin induced lung injury and fibrosis.Intratracheal bleomycin resulted in increased extracellular ATP levels in broncho-alveolar lavage (BAL) fluid in both wild type and CD39 deficient animals. However, ATP levels were significantly higher in CD39 deficient mice. In addition this was paralleled by increased inflammation and fibrosis in CD39 deficient animals, demonstrated by an increased number of BAL fluid inflammatory cells and pro-inflammatory cytokines, as well as by increased collagen deposition.In summary we were able to demonstrate that degradation of ATP in the extracellular space is crucial for the pathogenesis of pulmonary fibrosis.