Phenotypic abnormalities in long-term surviving cystic fibrosis mice

Pediatric Research

Volumn 40, Issue 2, 1996, Pages 233-241

  Kent, Geraldine ^a   Oliver, Mary ^a,f   Foskett, J Kevin ^a,b,g   Frndova, Helena ^a   Durie, Peter ^a,c   Forstner, Janet ^a,c   Forstner, Gordon G ^a,c   Riordan, John R ^a,c   Percy, Dean ^d   Buchwald, Manuel ^a,b,e  

^a HOSPITAL FOR SICK CHILDREN RESEARCH INSTITUTE   (Canada)

^b UNIVERSITY OF TORONTO   (Canada)

^c UNIVERSITY OF TORONTO   (Canada)

^d UNIVERSITY OF GUELPH   (Canada)

^e HOSPITAL FOR SICK CHILDREN   (Canada)

^f Cystic Fibrosis Foundation (US)   (United States)

^g Canadian Cystic Fibrosis Foundation   (Canada)

Author keywords

[No Author keywords available]

Indexed keywords

BUMETANIDE; CHLORIDE ION; CYCLIC AMP;

ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CONTROLLED STUDY; CYSTIC FIBROSIS; DIET THERAPY; FERTILITY; HISTOPATHOLOGY; INTESTINE EPITHELIUM; LUNG FUNCTION; MOUSE; NEWBORN; NONHUMAN; NOSE MUCOSA; PANCREAS FUNCTION; PHENOTYPE; PRIORITY JOURNAL; SURVIVAL RATE;

EID: 8944236549     PISSN: 00313998     EISSN: None     Source Type: Journal    
DOI: 10.1203/00006450-199608000-00008     Document Type: Article

References (30)

1. Rommens JM, Iannuzzi MC, Kerem B-S, Drumm ML, Melmer G, Dean M, Rozmahel R, Cole JL, Kennedy D, Hidaka N, Zsiga M, Buchwald M, Riordan JR, Tsui L-C, Collins FS 1989 Identification of the cystic fibrosis gene: chromosome walking and jumping. Science 245:1059-1065
2. Riordan, JR, Rommens JM, Kerem B-S, Alon N, Rozmahel R, Grzelczak Z, Zielenski J, Lok S, Plavsic N, Chou J-L, Drumm ML, Ianuzzi MC, Collins FS, Tsui L-C 1989 Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 245:1066-1073
3. Kerem B-S, Rommens JM, Buchanan JA, Markiewicz D, Cox TK, Chakravarti A, Buchwald M, Tsui L-C 1989 Identification of the cystic fibrosis gene: genetic analysis. Science 245:1073-1080
4. Quinton P 1990 Cystic fibrosis: a disease in electrolyte transport. FASEB J 4:2709-2717
5. Bear CE, Li C, Kartner N, Bridges RJ, Jensen TJ, Ramjeesingh M, Riordan JR 1992 Purification and functional reconstitution of the cystic fibrosis transmembrane regulator (CFTR). Cell 68:809-818
6. + transport in cystic fibrosis respiratory epithelia. Abnormal basal rate and response to adenylate cyclase activation. J Clin Invest 1986:1245-1252
7. Wood RE, Boat TF, Doershuk CF 1976 Cystic fibrosis. Am Rev Respir Dis 113:833-878
8. Welsh MJ, Tsui L-C, Boat TF, Beaudet AL 1994 Cystic fibrosis. In: Scriver CR, Beaudet AL, Sly WS, Valle D (eds) The Metabolic and Molecular Basis of Inherited Disease. McGraw-Hill, New York, pp 3799-3876
9. O'Neal WK, Beaudet AL 1994 Somatic gene therapy for cystic fibrosis. Hum Mol Genet 3:1497-1502
10. Snouwaert J, Brigman KK, Latour AM, Malouf NN, Boucher RC, Smithies O, Koller B 1992 An animal model for cystic fibrosis made by gene targeting. Science 257:1083-1008
11. Ratcliff R, Evans MJ, Cuthbert AW, MacVinish LJ, Foster D, Anderson JR, Colledge WH 1993 Production of a severe mutation in mice by gene targeting. Nat Genet 4:35-41
12. Dorin J, Dickinson P, Alton EWFW, Smith SN, Geddes DR, Stevenson BJ, Kimber WL, Fleming S, Clarke AR, Hooper ML, Anderson L, Beddington RSP, Porteous DJ 1992 Cystic fibrosis in the mouse by targeted insertional mutagenesis. Nature 359:211-215
13. O'Neal WK, Hasty P, McCray PB, Casey B, Rivera-Perez J, Welsh MJ, Beaudet AL, Bradley A 1993 A severe phenotype in mice with a duplication of exon 3 in the cystic fibrosis locus. Hum Mol Genet 2:1561-1569
14. LeSoef PN, England SJ, Bryan AC 1984 Passive respiratory mechanics in newborns and children. Am Rev Respir Dis 129:552-556
15. Cleghom GJ, Stringer DA, Forstner GG, Durie P 1986 Treatment of distal intestinal obstruction in patients with cystic fibrosis using a balanced intestinal lavage solution. Lancet 1:8-11
16. Specian RD, Neutra MR 1980 Mechanism of rapid mucus secretion in goblet cells stimulated with acetylcholine. J Cell Biol 85:626-640
17. Trier JS 1966 The Paneth cells: an enigma. Gastroenterology 51:560-562
18. Sandow MJ, Whitehead R 1979 Progress report: the Paneth cell. Gut 20:420-431
19. Banwell JG, Howard R, Kabir I, Adrian TE, Diamond RH, Abramoswsky C 1993 Small intestinal growth caused by feeding red kidney bean phytohemagglutinin lectin to rats. Gastroenterology 104:315-324
20. Gordon JI 1993 Understanding gastrointestinal epithelial cell biology: lessons from mice with help from worms and flies. Gastroenterology 104:315-324
21. Madara JL, Trier JS 1987 Functional morphology of the mucosa of the small intestine. In: Johnson LR (ed) Physiology of the Intestinal Tract, Vol 1, 2nd Ed. Reven Press, New York, pp 1209-1249
22. Pearce AGE 1968 Histochemistry: Theoretical and Applied. Little, Brown Co., Boston
23. Wesley A, Forstner JR, Qureshi R, Mantle M, Forstner G 1983 Human intestinal mucin in cystic fibrosis. Pediatr Res 17:65-69
24. Cheng P, Boat TF, Cranfill K, Yankaskas JR, Boucher RC 1989 Increased sulfation of glycoconjugates by cultured nasal epithelial cells from patients with cystic fibrosis. J Clin Invest 84:68-72
25. Dosanj A, Lencer W, Brown D, Ausiello DA, Stow JL 1994 Heterologous expression of ΔF508 CFTR results in decreased sialylation of membrane glycoconjugates. Am J Physiol 266:C360-C366
26. Thornton DJ, Sheehan JK, Carlstedt I 1991 Heterogeneity of mucus glycoproteins from cystic fibrosis sputum. Biochem J 276:677-682
27. Clarke LL, Grubb BR, Gabriel SE, Smithies O, Koller BH, Boucher RC 1992 Defective epithelial chloride transport in a gene-targeted mouse model of cystic fibrosis. Science 257:1125-1128
28. Whitsett J, Dey CR, Stripp BR, Wikenheiser KA, Clark JC, Wert SE, Gregory RJ, Smith AE, Cohn JA, Wilson JM, Engelhardt J 1992 Human cystic fibrosis transmembrane conductance regulator directed to respiratory epithelial cells of transgenic mice. Nat Genet 2:13-20
29. Engelhardt JF, Yankaskas JR, Ernst SA, Yang Y, Marino CR, Boucher RC, Cohn JA, Wilson JM 1992 Submucosal glands are the predominant site of CFTR expression in the human bronchus. Nat Genet 2:240-248
30. Jacquot J, Puchelle E, Hinnrasky J, Fuchey C, Bettinger C, Spilmont C, Bonnet N, Dieterle A, Dreyer D, Pavirani A, Dalemans W 1993 Localization of the cystic fibrosis transmembrane conductance regulator in airway secretory glands. Eur Respir J 6:169-176