Article
A mouse model for the delta F508 allele of cystic fibrosis.
The Journal of clinical investigation - 1 Oct 1995
Zeiher B G, Eichwald E, Zabner J, Smith J J, Puga A P, McCray P B, Capecchi M R, Welsh M J, Thomas K R
Abstract excerpt
The most common cause of cystic fibrosis is a mutation that deletes phenylalanine 508 in cystic fibrosis transmembrane conductance regulator (CFTR). The delta F508 protein is misprocessed and degraded rather than traveling to the apical membrane. We used a novel strategy to introduce the delta F508 mutation into the mouse CFTR gene. Affected epithelia from homozygous delta F508 mice lacked CFTR in the apical...
Topics
- Alleles
- Animals
- Base Sequence
- Cystic Fibrosis
- Cystic Fibrosis Transmembrane Conductance Regulator
- Digestive System
- Disease Models, Animal
- Electrolytes
- Humans
- Mice
- Mice, Inbred C57BL