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...
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