PT - JOURNAL ARTICLE AU - Colin Bingle AU - Kirsty Wilson AU - Marcus Mall AU - Doris Rassl AU - Lynne Bingle TI - Increased epithelial production of LPLUNC1 in cystic fibrosis lung disease DP - 2011 Sep 01 TA - European Respiratory Journal PG - p3086 VI - 38 IP - Suppl 55 4099 - http://erj.ersjournals.com/content/38/Suppl_55/p3086.short 4100 - http://erj.ersjournals.com/content/38/Suppl_55/p3086.full SO - Eur Respir J2011 Sep 01; 38 AB - Members of the PLUNC family of secreted proteins have been implicated in innate defence of the upper airways and nasopharynx. Although array data suggest that they are differentially expressed in some lung diseases validation has not been forthcoming. We previously showed that SPLUNC1 is increased in severe cystic fibrosis (CF). In the present study we have investigated the expression of LPLUNC1 in severe CF and studied both proteins in mouse models of CF lung disease. There was marked epithelial staining of LPLUNC1 in diseased small airways and submucosal glands. in CF, similar to our previous data with SPLUNC1. The two proteins are not co-expressed in the CF lung as LPLUNC1 is co-localised with MUC5A/C in goblet cells, whereas SPLUNC1 is present in a non-ciliated, non-goblet cell population. Expression of both proteins was unchanged (and very limited), in CFTR knockout mice. However, in CCSP-betaENaC transgenic mice, a model for CF lung disease, there was very strong staining of both proteins in the airways and in the luminal contents. This was most marked for lplunc1 and was noted within 2 weeks of birth. As in CF, the two proteins are present in distinct cells within the epithelium. Lplunc1 was absent from the lavage fluid of non-transgenics, in keeping with the lack of goblet cells, but was readily detected in transgenics. Our results suggest that alterations in expression of these putative innate immune molecules is associated with CF lung disease in both humans and mice. It remains unclear if this elevation of protein production, which results from phenotypic alteration of the cells within the diseased epithelium, plays a role in the pathogenesis of the disease.