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AN ANIMAL-MODEL FOR CYSTIC-FIBROSIS MADE BY GENE TARGETING
Author(s): SNOUWAERT JN, BRIGMAN KK, LATOUR AM, MALOUF NN, BOUCHER RC, SMITHIES O, KOLLER BH
Source: SCIENCE    Volume: 257    Issue: 5073    Pages: 1083-1088    Published: AUG 21 1992  
Times Cited: 523     References: 25     
Abstract: Cystic fibrosis results from defects in the gene encoding a cyclic adenosine monophosphate-dependent chloride ion channel known as the cystic fibrosis transmembrane conductance regulator (CFTR). To create an animal model for cystic fibrosis, mice were generated from embryonic stem cells in which the CFTR gene was disrupted by gene targeting. Mice homozygous for the disrupted gene display many features common to young human cystic fibrosis patients, including failure to thrive, meconium ileus, alteration of mucous and serous glands, and obstruction of glandlike structures with inspissated eosinophilic material. Death resulting from intestinal obstruction usually occurs before 40 days of age.
Document Type: Article
Language: English
Addresses:
1. UNIV N CAROLINA, DEPT MED, CHAPEL HILL, NC 27599 USA
2. UNIV N CAROLINA, DEPT PATHOL, CHAPEL HILL, NC 27599 USA
Publisher: AMER ASSOC ADVANCEMENT SCIENCE, 1200 NEW YORK AVE, NW, WASHINGTON, DC 20005
Subject Category: Multidisciplinary Sciences
IDS Number: JJ884
ISSN: 0036-8075
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