ABCMdb

PMID: 7491981

Eckman EA, Cotton CU, Kube DM, Davis PB
Dietary changes improve survival of CFTR S489X homozygous mutant mouse.
Am J Physiol. 1995 Nov;269(5 Pt 1):L625-30., [PubMed]

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0 ABCC7 p.Ser489* 269:L625-L630, 1995. ;Am J Physiol Lung Cell Mol Physiol E. A. Eckman, C. U. Cotton, D. M. Kube and P. B. Davis homozygous mutant mouse Dietary changes improve survival of CFTR S489X You might find this additional info useful... 25 other HighWire-hosted articles:This article has been cited by http://ajplung.physiology.org/content/269/5/L625#cited-by including high resolution figures, can be found at:Updated information and services http://ajplung.physiology.org/content/269/5/L625.full can be found at:Molecular Physiology American Journal of Physiology - Lung Cellular andaboutAdditional material and information http://www.the-aps.org/publications/ajplung This information is current as of October 25, 2012. Login to comment 6 ABCC7 p.Ser489* It is published 12 times a year (monthly) by the American covering the broad scope of molecular, cellular, and integrative aspects of normal and abnormal function of publishes original researchAmerican Journal of Physiology - Lung Cellular and Molecular Physiology Dietary changes improve survival of CFTR S489X homozygous mutant mouse ELIZABETH A. ECKMAN, CALVIN U. COTTON, DIANNE M. KUBE, AND PAMELA B. DAVIS Departments of Pediatrics, Molecular Biology and Microbiology, and Physiology and Biophysics, Case Western Reserve University, Cleveland, Ohio 44106 E&man, Elizabeth A., Calvin U. Cotton, Dianne M. Kube, and Pamela B. Davis. Login to comment 7 ABCC7 p.Ser489* Dietary changes improve survival of CFTR S489X homozygous mutant mouse. Login to comment 12 ABCC7 p.Ser489* 13): L625-L630,1995.- Over 90% of untreated CFTR S489X homozygous (CF) mutant mice reportedly die of intestinal obstruction by 40 days of age, significantly limiting their usefulness as a model for the human disease. Login to comment 24 ABCC7 p.Ser489* Snouwaert et al. [lo; University of North Carolina (UNC)] and Ratcliff et al. (7; University of Cambridge) designed targeting constructs to replace a portion of the CFTR gene with two copies of the neomycin resistance gene (S489X mutation) or a hypoxanthine phosphoribosyl transferase (HPRT) mini-gene, respectively. Login to comment 40 ABCC7 p.Ser489* When S489X CFTR (-/-) mice are fed Peptamen complete elemental liquid diet and bedded on corn cob pellets instead of the traditional wood shavings, 88% of these animals which are alive at 10 days of age survive to maturity. Login to comment 43 ABCC7 p.Ser489* UNC mice heterozygous for the S489X mutation (CFTR + /-> were purchased from the Jackson Laboratories 1040-0605/95 $3.00 Copyright o 1995 the American Physiological Society L625 (Bar Harbor, ME). Login to comment 54 ABCC7 p.Ser489* Primers III and IV were used to amplify the S489X neo-disrupted allele. Login to comment 57 ABCC7 p.Ser489* Both wild-type CFTR and S489X alleles were amplified in a single reaction. Login to comment 60 ABCC7 p.Ser489* Expected product sizes were N 500 base pairs (bp) for the wild-type allele (primers I and II) and 650 bp for the S489X allele (primers III and IV). Login to comment 129 ABCC7 p.Ser489* DISCUSSION The UNC S489X homozygous CF mice have a severe phenotype resulting from complete inactivation of the murine CFTR gene. Login to comment 148 ABCC7 p.Ser489* Indeed, a few of the S489X homozygous mice placed on this regimen actually succumbed to intestinal obstruction, so it is possible that the survivors suffered intermittent bouts of this complication and consequently were runts. Login to comment 151 ABCC7 p.Ser489* S489X CF knockout mice transgenic for human CFTR under control of the rat intestinal fatty acid-binding gene promoter express CFTR predominantly, but not exclusively, in the intestine and are protected against death from intestinal obstruction even without special diet or Forskolin JI + CFTR (+/&) Solid Food -O- CFTR (+/&) Peptamen +I+ CFTR (4) Peptamen -6 4 -2 0 2 4 6 8 10 Time (min) Fig. 4. Login to comment