[Truncated abstract] Cystic fibrosis (CF) is an autosomal recessive disorder that affects millions of individuals worldwide. It is the most common life-shortening condition that affects children. Approximately 1 in every 2,800 babies born in Australia every year are diagnosed with CF. The disease is caused by mutations in the CF gene resulting in abnormal cystic fibrosis transmembrane conductance regulator (CFTR) function at the epithelial membranes of many organ systems, most importantly in the respiratory system. Lung failure characterised by early neutrophilic inflammation, chronic bacterial infection and airway obstruction, is the most significant cause of morbidity and mortality among patients with CF. Although inflammation and infections occurs early in the course of CF lung disease, little is known regarding the contribution of the airway epithelium to this excessive inflammatory response. This conundrum is partially due to the limitations of suitable models for the study of dysregulated pathways in the CF airway epithelium. Therefore, this study tests the following hypotheses. Firstly, that the airway epithelium in CF is inherently pro-inflammatory compared to the healthy epithelium. Secondly, that respiratory viral infections, specifically human rhinoviruses (HRVs), trigger inflammatory responses in the CF airway epithelium. Thirdly, that HRV-induced effects observed in the CF airway epithelium can be ameliorated...
|Qualification||Doctor of Philosophy|
|Publication status||Unpublished - 2012|