Animal studies of cystic fibrosis.
Cystic fibrosis is the most common life-threatening autosomal recessive genetic disorder in Caucasian populations. It is a disease primarily of epithelial tissues, including the airway, pancreatic duct, intestine, genital tract and sweat glands. The affected gene was cloned and characterized in 1989. In the absence of an identified natural animal model of the disease, a major effort has been made to develop transgenic cystic fibrosis mice, by disrupting the gene in these laboratory animals. Such mice show many, but not all, of the symptoms of cystic fibrosis. In this article, the major past and present contributions of other animal systems to our understanding of cystic fibrosis are examined and their potential for future studies of this disease are discussed. It is intended to give the reader a broad overview of the field, exploring the usefulness of animal studies, rather than dealing more fully with specific aspects of cystic fibrosis.
Tebbutt
S J
SJ
Department of Biochemistry, University of Otago, Dunedin, New Zealand. tebbutts@sanger.otago.ac.nz
D000818:Animals; D002874:Chromosome Mapping; D002897:Chromosomes, Human, Pair 7; D003550:Cystic Fibrosis / Q000235:genetics / Q000503:physiopathology* / Q000628:therapy; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000096:biosynthesis / Q000235:genetics; D004195:Disease Models, Animal; D005808:Genes, Recessive; D015316:Genetic Therapy; D006801:Humans; D051379:Mice; D008822:Mice, Transgenic; D044465:White People
C505032:CFTR protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator