Article
Generation and characterization of a delta F508 cystic fibrosis mouse model.
Nature genetics - 1 Aug 1995
Colledge W H, Abella B S, Southern K W, Ratcliff R, Jiang C, Cheng S H, MacVinish L J, Anderson J R, Cuthbert A W, Evans M J
Abstract excerpt
We have generated mice carrying the most common mutation in cystic fibrosis (CF), delta F508, within the cystic fibrosis (Cftr) gene. Mutant animals show pathological and electrophysiological changes consistent with a CF phenotype. delta F508-/- mice die from peritonitis and show deficiencies in cAMP-activated electrogenic Cl- transport. These mice produce delta F508 transcripts and show the temperature-dependent...
Topics
- Animals
- Base Sequence
- Cells, Cultured
- Chloride Channels
- Cystic Fibrosis
- Cystic Fibrosis Transmembrane Conductance Regulator
- DNA
- Disease Models, Animal
- Electrophysiology
- Membrane Proteins