As BPIF proteins have been increasingly implicated in the pathogenesis of CF (Garcia-Caballero et al. 2009; Roxo-Rosa et al. 2006; McCray et al. 2005; Bingle et al. 2007) we undertook to study BPIFB1 in both diseased human tissue and in an established mouse model of CF. This evidence concerns the gene BPIFB1 and cystic fibrosis.