|Cystic fibrosis mouse models|
Actually, we are using two mouse models for Cystic Fibrosis for our research targets. The first was established in 1992 by Dorin and colleagues by targeted mutation using an insertional gene targeting vector to disrupt exon 10 of the murine Cftr gene in 129P2 embryonic stem cells. Since the mutation in these CftrTgH(neoim)Hgu mice is slightly ´leaky´, mice produce wild type Cftr mRNA in low levels (Dorin et al., 1994). Therefore, animals do not suffer from the severe gastrointestinal phenotype many CF mouse models do, but nevertheless display the electrophysiological defect in the gastrointestinal and respiratory tract which is characteristic of CF (Smith et al., 1994). In cooperation with the Institute for Laboratory Animal Science of Hannover Medical School CftrTgH(neoim)Hgu mice were backcrossed from their mixed genetic background on inbred strain C57Bl/6JZtm and established as congenic B6.129P2(CF/3)-CftrTgH(neoim)Hgu mice (Charizopoulou et al., 2004, 2006).
Dorin JR et al. (1992).
Cystic Fibrosis in the mouse by targeted insertional mutagenesis.
Nature 359, 211−215.
Dorin JR et al. (1994).
Long-term survival of the exon 10 insertional cystic fibrosis mutant mouse is a consequence of low level residual wild-type Cftr gene expression.
Mammalian Genome 5, 465−472.
Smith SN et al. (1995).
Bioelectric characteristics of exon 10 insertional cystic fibrosis mouse: comparison with humans.
Am J Physiol 268:C297−C307.