TY - JOUR T1 - Distribution of WFDC2/HE4, a putative host defence protein, in the respiratory tract of normal and diseased mice JF - European Respiratory Journal JO - Eur Respir J VL - 42 IS - Suppl 57 SP - P3886 AU - Olympia Gianfrancesco AU - Lynne Bingle AU - Colin Bingle Y1 - 2013/09/01 UR - http://erj.ersjournals.com/content/42/Suppl_57/P3886.abstract N2 - WFDC2/HE4 encodes a poorly characterised secretory protein that shares structural similarity to the multifunctional host defence protein, SLPI, through possession of two conserved Whey Acidic Protein/four disulphide-core (WFDC) domains. Like SLPI, WFDC2 is expressed in multiple epithelia within the respiratory tract and although its’ function remains unresolved, recent evidence supports its' role as an antiproteinase. We have previously shown that it is over-expressed in lung diseases characterised by abnormalities of protease/antiprotease balance, including cystic fibrosis. Nothing is known about the distribution of WFDC2 in the mouse. In this study we have systematically localized the protein in normal adult mice as well in a number of mouse models characterised by epithelial remodelling. Strong WFDC2 staining is seen throughout the airway epithelium from the trachea down to the smallest airways. Staining is also seen in tracheal submucosal glands. No staining is seen in the peripheral lung. Interestingly, staining is absent from the respiratory epithelium of the nasal passages whereas goblet cells stain strongly in these locations. In bleomycin and OVA treated mice there is elevated staining throughout the remodeled epithelium that is not limited to goblet cells and strikingly there is very strong staining within the alveolar (Type II) cells in these regions. Mouse WFDC2 is elevated in a number of chronic disease models and suggests that the gene exhibits a level of promiscuity relating to its molecular regulation. Knowledge of the complex expression pattern of this protein will allow the use of tractable mouse models of disease to dissect its’ function. ER -