Project description:In Cystic Fibrosis (CF) patients, cycles of infection and inflammation lead to fatal lung damage. Here, we made use of a CF pig model to investigate proteome alterations in lung, lung-derived leukocytes and PBMCs.
Project description:<p>The major goal of this project is to apply second generation resequencing technology to identify disease causing variants influencing pediatric and adult lung diseases in a collection of two longitudinal population cohorts of cystic fibrosis patients that have been well characterized for a comprehensive set of clinical traits. In Phase I, exome sequencing was performed on 43 cystic fibrosis patients with early <i>Pa</i> infection and 48 cystic fibrosis patients with late <i>Pa</i> infection to identify variants influencing the time to onset of <i>Pa</i> infection. In Phase II, additional exomes were added to the study, to reach a total of 91 individuals with early <i>Pa</i> infection and 96 with late <i>Pa</i> infection. The majority of the 340 subjects of Phase II do not have a <i>Pa</i> infection phenotype, but instead have a pulmonary function phenotype (121 severe vs. 124 mild impairment) as determined by the survival corrected Kulich FEV percentile of Corey et al. A small minority have intermediate phenotypes and/or show severe decline in lung function during childhood.</p>