Residual cftr expression varies with age in cftrtm1Hgu cystic fibrosis mice: Impact on morphology and physiology

Pathobiology

Volumn 70, Issue 2, 2002, Pages 89-97

  Larbig, M ^a   Jansen, S ^a   Dorsch, M ^a   Bernhard, W ^a   Bellmann, B ^a   Dorin, J R ^b   Porteous, D J ^b   Von der Hardt, H ^a   Steinmetz, I ^a   Hedrich, H J ^a   Tuemmler, B ^a   Tschernig, Thomas ^a  

^a HANNOVER MEDICAL SCHOOL   (Germany)

^b WESTERN GENERAL HOSPITAL   (United Kingdom)

Author keywords

Alveolar clearance; CFTR; Cystic fibrosis; Lung pathology; Surfactant; Transgenic mouse disease model

Indexed keywords

LUNG SURFACTANT; MESSENGER RNA; OXIDE; PHOSPHOLIPID; SCANDIUM; TRANSMEMBRANE CONDUCTANCE REGULATOR;

AGING; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BODY WEIGHT; BRONCHUS; COMPARATIVE STUDY; CONTROLLED STUDY; CYSTIC FIBROSIS; EXON; EXPERIMENTAL MODEL; GENE INSERTION; GENE MUTATION; HOMEOSTASIS; INTESTINE; KIDNEY; KIDNEY EPITHELIUM; LIFESPAN; LUNG; LUNG CLEARANCE; MORPHOLOGY; MOUSE; NONHUMAN; PATHOPHYSIOLOGY; PHYSIOLOGY; PRIORITY JOURNAL; PROTEIN DETERMINATION; PROTEIN EXPRESSION; PROTEIN LOCALIZATION; RESPIRATORY EPITHELIUM; RESPIRATORY SYSTEM; TISSUE DISTRIBUTION; WEIGHT GAIN;

AGING; ANIMALS; BODY WEIGHT; BRONCHOALVEOLAR LAVAGE FLUID; CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; INTESTINES; KIDNEY; LUNG; MICE; MICE, INBRED CFTR; MODELS, ANIMAL; MUCOCILIARY CLEARANCE; MUTATION; PHOSPHOLIPIDS; REVERSE TRANSCRIPTASE POLYMERASE CHAIN REACTION; RNA, MESSENGER;

MUS MUSCULUS;

EID: 12244277884     PISSN: 10152008     EISSN: None     Source Type: Journal    
DOI: 10.1159/000067308     Document Type: Article

References (49)

1. Welsh MJ, Tsui LC, Boat TF, Beaudet AL: Cystic fibrosis; in Scriver CR, Beaudet AL, Sly WS, Valle D (eds): The Metabolic Basis of Inherited Disease. New York, McGraw-Hill, 1995, pp 3799-3876.
2. Cressman VL, Hicks EM, Funkhauser WK, Backlund DC, Koller BH: The relationship of chronic mucin secretion to airway disease in normal and cftr-deficient mice. Am J Respir Cell Mol Biol 1998;19:853-866.
3. Snouwaert JN, Brigman KK, Latour AM, Iraj E, Schwab U, Gilmour MI, Koller BH: A murine model of cystic fibrosis. Am J Respir Crit Care Med 1995;151:59-64.
4. Smith SN, Steel DM, Middleton PG, Munkonge FM, Geddes DM, Caplan NJ, Porteous DJ, Dorin JR, Alton EW: Bioelectric characteristics of exon 10 insertional cystic fibrosis mouse: Comparison with humans. Am J Physiol 1995;268:C297-C307.
5. Dorin JR: Development of mouse models for cystic fibrosis. J Inherit Metab Dis 1995;18:495-500.
6. Zahm J-M, Gaillard D, Dupuit F, Hinnrasky J, Porteous DJ, Dorin JR, Puchelle E: Early alterations in mucociliary clearance and inflammation of the lamina propria in CF mice. Am J Physiol 1997;272:C853-C859.
7. Davidson DJ, Dorin JR, McLachlan G, Ranaldi V, Lamb D, Doherty C, Govan J, Porteous DJ: Lung disease in the cystic fibrosis mouse exposed to bacterial pathogens. Nat Genet 1995;9:351-357.
8. Dorin JR, Dickinson P, Alton EW, Smith SN, Geddes DM, Stevenson BJ, Kimber WL, Fleming S, Clarke AR, Hooper ML, Anderson L, Beddington RSP, Porteous DJ: Cystic fibrosis in the mouse by targeted insertional mutagenesis. Nature 1992;359:211-215.
9. Dorin JR, Stevenson BJ, Fleming S, Alton EW, Dickinson P, Porteous DJ: Long-term survival of the exon 10 insertional cystic fibrosis mutant mouse is a consequence of low level residual wild-type cftr gene expression. Mamm Genome 1994;5:465-472.
10. Nicklas W, Baneux P, Boot R, Decelle T, Deeny AA, Fumanelli M, Illgen-Wilcke B: Recommendations for the health monitoring of rodent and rabbit colonies in breeding experimental units. Recommendations of the Federation of European Laboratory Animal Science Associations (FELASA) Working Group. Lab Anim 2002;36:20-42.
11. Chomczynski P, Sacchi N: Single-step method of RNA isolation by acid guanidinium thiocyanate-phenol-chloroform extraction. Anal Biochem 1987;162:156-159.
12. Hoof T, Riordan JR, Tümmler B: Quantitation of mRNA by the kinetic polymerase chain reaction assay: A tool for monitoring P-glycoprotein gene expression. Anal Biochem 1991;196:161-169.
13. Yorifuji T, Lemna WK, Ballard CF, Rosenbloom CL, Rozmahel R, Plavsic N, Tsui LC, Beaudet AL: Molecular cloning and sequence analysis of the murine cDNA for the cystic fibrosis transmembrane conductance regulator. Genomics 1991;10:547-550.
14. t1neoHgu/t1neoHgu mice. Thorax 1997;52:723-730.
15. Bligh EG, Dyer WJ: A rapid method of total lipid extraction and purification. Can J Biochem Physiol 1959;37:911-917.
16. Folch J, Lees M, Stanley GHS: A simple method for the isolation and purification of total lipids from animal tissue. J Biol Chem 1957;226:497-509.
17. Bartlett GR: Phosphorus assay in column chromatography. J Biol Chem 1959;234:466-468.
18. Bellmann B, Muhle H, Creutzenberg O, Dasenbrock C, Kilpper R, MacKenzie JC, Morrow P, Mermelstein R: Lung clearance and retention of toner, utilizing a tracer technique, during chronic inhalation exposure in rats. Fundam Appl Toxicol 1991;17:300-313.
19. Muhle H, Bellmann B, Heinrich U: Overloading of lung clearance during chronic exposure of experimental animals to particles. Ann Occup Hyg 1988;32:141-147.
20. Festing MF, Blackmore DK: Life span of specified-pathogen-free (MRC category 4) mice and rats. Lab Anim 1971;5:179-192.
21. tm1Hgu/tm1Hgu mice compared to MF-1 mice. Exp Lung Res 2001;27:349-366.
22. Snouwaert JN, Brigman KK, Latour AM, Malouf NN, Boucher RC, Smithies O, Koller BH: An animal model for cystic fibrosis made by gene targeting. Science 1992;257:1083-1088.
23. Highsmith WE, Burch LH, Zhou Z, Olsen JC, Boat TE, Spock A, Gorvoy JD, Quittel L, Friedman KJ, Silverman LM: A novel mutation in the cystic fibrosis gene in patients with pulmonary disease but normal sweat chloride concentrations. N Engl J Med 1994;331:974-980.
24. Highsmith WE Jr, Burch LH, Zhou Z, Olsen JC, Strong TV, Smith T, Friedman KJ, Silverman LM, Boucher RC, Collins FS, Knowles MR: Identification of a splice site mutation (2789 + 5 G > A) associated with small amounts of normal CFTR mRNA and mild cystic fibrosis. Hum Mutat 1997;9:332-338.
25. Chiba-Falek O, Kerem E, Shoshani T, Aviram M, Augarten A, Bentur L, Tal A, Tullis E, Rahat A, Kerem B: The molecular basis of disease variability among cystic fibrosis patients carrying the 3849 + 10 kb C → T mutation. Genomics 1998;53:276-283.
26. Chiba-Falek O, Parad RB, Kerem E, Kerem B: Variable levels of normal RNA in different fetal organs carrying a cystic fibrosis transmembrane conductance regulator splicing mutation. Am J Respir Crit Care Med 1999;159:1998-2002.
27. Teng H, Jorissen M, van Poppel H, Legius E, Cassiman JJ, Cuppens H: Increased proportion of exon 9 alternatively spliced CFTR transcripts in vas deferens compared with nasal epithelial cells. Hum Mol Genet 1997;6:85-90.
28. Borthwick DW, West JD, Keighren MA, Flockhart JH, Innes BA, Dorin JR: Murine submucosal glands are clonally derived and show a cystic fibrosis gene-dependent distribution pattern. Am J Respir Cell Mol Biol 1999;20:1181-1189.
29. Geiser M, Zimmermann B, Baumann M, Cruz-Orive LM: Does lack of cftr gene lead to developmental abnormalities in the lung? Exp Lung Res 2000;26:551-564.
30. Gallagher AM, Gottlieb RA: Proliferation, not apoptosis, alters epithelial cell migration in small intestine of cftr null mice. Am J Physiol Gastrointest Liver Physiol 2001;281:G681-G687.
31. Larson JE, Delcarpio JB, Farberman MM, Morrow SL, Cohen JC: CFTR modulates lung secretory cell proliferation and differentiation. Am J Physiol Lung Cell Mol Physiol 2000;279:L333-L341.
32. Harvey B-G, Crystal RG: Pulmonary responses to chronic inorganic dust exposure; in Crystal RG, West JB, Weibel ER, Barnes PJ (eds): The Lung. Philadelphia, Lippincott-Raven, 1997, pp 2339-2352.
33. Deneuville E, Perrot Minot C, Pennaforte F, Roussey M, Zahm JM, Clavel C, Puchelle E, De Bentzmann S: Revisited physicochemical and transport properties of respiratory mucus in genotyped cystic fibrosis patients. Am J Respir Crit Care Med 1997;156:166-172.
34. Engelhardt JF, Zepeda M, Cohn JA, Yankaskas JR, Wilson JM: Expression of the cystic fibrosis gene in adult human lung. J Clin Invest 1993;93:737-749.
35. Bernhard W, Haagsman HP, Tschernig T, Poets CF, Postle AD, van Eijk ME, von der Hardt H: Conductive airway surfactant: Surface-tension function, biochemical composition, and possible alveolar origin. Am J Respir Cell Mol Biol 1997;17:41-50.
36. Chao W, Spragg RG, Smith RM: Inhibitory effect of porcine surfactant on the respiratory burst oxidase in human neutrophils. Attenuation of p47phox and p67phox membrane translocation as the mechanism. J Clin Invest 1995;96:2654-2660.
37. Crouch E, Wright JR: Surfactant proteins a and d and pulmonary host defense. Annu Rev Physiol 2001;63:521-554.
38. Gosselin D, Stevenson MM, Cowley EA, Griesenbach U, Eidelman DH, Boule M, Tam MF, Kent G, Skamene E, Tsui LC, Radzioch D: Impaired ability of cftr knockout mice to control lung infection with Pseudomonas aeruginosa. Am J Respir Crit Care Med 1998;157:1253-1262.
39. Heeckeren A, Walenga R, Konstan MW, Bonfield T, Davis PB, Ferkol T: Excessive inflammatory response of cystic fibrosis mice to bronchopulmonary infection with Pseudomonas aeruginosa. J Clin Invest 1997;100:2810-2815.
40. McCray PB Jr, Zabner J, Jia HP, Welsh MJ, Thorne PS: Efficient killing of inhaled bacteria in DeltaF508 mice: Role of airway surface liquid composition. Am J Physiol 1999;277:L183-L190.
41. Pier GB, Grout M, Zaidi TS, Goldberg JB: How mutant CFTR may contribute to Pseudomonas aeruginosa infection in cystic fibrosis. Am J Respir Crit Care Med 1996;154:S175-S182.
42. -/- mice. Infect Immun 2001;69:5138-5150.
43. Dickinson P, Kimber WL, Kilanowski FM, Webb S, Stevenson BJ, Porteous DJ, Dorin JR: Enhancing the efficiency of introducing precise mutations into the mouse genome by hit and run gene targeting. Transgenic Res 2000;9:55-66.
44. Kent G, Iles R, Bear CE, Huan LJ, Griesenbach U, McKerlie C, Frndova H, Ackerley C, Gosselin D, Radzioch D, O'Brodovich H, Tsui LC, Buchwald M, Tanswell AK: Lung disease in mice with cystic fibrosis. J Clin Invest 1997;100:3060-3069.
45. Rozmahel R, Wilschanski M, Matin A, Plyte S, Oliver M, Auerbach W, Moore A, Forstner J, Durie P, Nadeau J, Bear C, Tsui LC: Modulation of disease severity in cystic fibrosis transmembrane conductance regulator deficient mice by a secondary genetic factor. Nat Genet 1996;12:280-287.
46. Madsen J, Kliem A, Tornoe I, Skjodt K, Koch C, Holmskov U: Localization of lung surfactant protein D on mucosal surfaces in human tissues. J Immunol 2000;164:5866-5870.
47. Postle AD, Mander A, Reid KB, Wang JY, Wright SM, Moustaki M, Warner JO: Deficient hydrophilic lung surfactant proteins A and D with normal surfactant phospholipid molecular species in cystic fibrosis. Am J Respir Cell Mol Biol 1999;20:90-98.
48. Armstrong DS, Grimwood K, Carlin JB, Carzino R, Gutierrez JP, Hull J, Olinsky A, Phelan EM, Robertson CF, Phelan PD: Lower airway inflammation in infants and young children with cystic fibrosis. Am J Respir Crit Care Med 1997;156:1197-1204.
49. Bronsveld I, Mekus F, Bijman J, Ballmann M, de Jonge HR, Laabs U, Halley DJ, Ellemunter H, Mastella G, Thomas S, Veeze HJ, Tümmler B: Chloride conductance and genetic background modulate the cystic fibrosis phenotype of delta F508 homozygous twins and siblings. J Clin Invest 2001;108:1705-1715.