Cystic fibrosis mice carrying the missense mutation G551D replicate human genotype-phenotype correlations.

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Article

Cystic fibrosis mice carrying the missense mutation G551D replicate human genotype-phenotype correlations.

The EMBO journal - 1 Mar 1996

Delaney S J, Alton E W, Smith S N, Lunn D P, Farley R, Lovelock P K, Thomson S A, Hume D A, Lamb D, Porteous D J, Dorin J R, Wainwright B J

Abstract excerpt

We have generated a mouse carrying the human G551D mutation in the cystic fibrosis transmembrane conductance regulator gene (CFTR) by a one-step gene targeting procedure. These mutant mice show cystic fibrosis pathology but have a reduced risk of fatal intestinal blockage compared with 'null' mut...

Topics

Animals
Animals, Newborn
Base Sequence
Chlorides
Cystic Fibrosis
Cystic Fibrosis Transmembrane Conductance Regulator
DNA Primers
Disease Models, Animal
Electrophysiology
Gene Targeting
Genotype

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Humans

Intestinal Obstruction

Ion Transport

Meconium

Mice

Mice, Transgenic

Molecular Sequence Data

Source

Open in PubMed Open full text

Identifiers

PMID: 8605891
PMCID: PMC449990

Genes

CFTR

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 30 Aug 2026. Prepared record publication-8605891-2026-08-30.1. Manifest publication-graph-cohort-2026-08-30.1.

Source receipt: PubMed via Helena Literature Corpus, retrieved 30 Aug 2026.

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