Cystic fibrosis mice carrying the missense mutation G551D replicate human genotype-phenotype correlations.
Delaney SJ, Alton EW, Smith SN, Lunn DP, Farley R, Lovelock PK, Thomson SA, Hume DA, Lamb D, Porteous DJ, Dorin JR, Wainwright BJ.
Delaney SJ, et al. Among authors: farley r.
EMBO J. 1996 Mar 1;15(5):955-63.
EMBO J. 1996.
PMID: 8605891
Free PMC article.
The G551D mutant mice show greatly reduced CFTR-related chloride transport, displaying activity intermediate between that of cftr(mlUNC) replacement ('null') and cftr(mlHGU) insertional (residual activity) mutants and equivalent to approximately 4% of wild-type CFTR activity. The …
The G551D mutant mice show greatly reduced CFTR-related chloride transport, displaying activity intermediate between that of cftr(mlUNC) rep …