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Volumn 13, Issue 4, 2003, Pages 253-258

Basic science of cystic fibrosis

Author keywords

Cystic fibrosis; Diagnosis; Genetics; Genotype; Phenotype pathogenesis

Indexed keywords

CHLORIDE; DNA; ELASTASE; GENE PRODUCT; MESSENGER RNA; NITRIC OXIDE; SODIUM; TRANSMEMBRANE CONDUCTANCE REGULATOR;

EID: 0037635437     PISSN: 09575839     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0957-5839(03)00029-0     Document Type: Conference Paper
Times cited : (1)

References (30)
  • 1
    • 0024453308 scopus 로고
    • Identification of the cystic fibrosis gene: Chromosome walking and jumping
    • Rommens J M, Iannuzzi M C, Kerem B et al. Identification of the cystic fibrosis gene: chromosome walking and jumping. Science 1989; 245: 1059-1065.
    • (1989) Science , vol.245 , pp. 1059-1065
    • Rommens, J.M.1    Iannuzzi, M.C.2    Kerem, B.3
  • 2
    • 0035406227 scopus 로고    scopus 로고
    • Recent advances in cystic fibrosis
    • Doull I J. Recent advances in cystic fibrosis. Arch Dis Child 2001; 85: 62-66.
    • (2001) Arch Dis Child , vol.85 , pp. 62-66
    • Doull, I.J.1
  • 3
    • 0037027912 scopus 로고    scopus 로고
    • An overview of the pathogenesis of cystic fibrosis lung disease
    • Boucher R C. An overview of the pathogenesis of cystic fibrosis lung disease. Adv Drug Deliv Rev 2002; 54: 1359-1371.
    • (2002) Adv Drug Deliv Rev , vol.54 , pp. 1359-1371
    • Boucher, R.C.1
  • 5
    • 0037157787 scopus 로고    scopus 로고
    • Genotype-phenotype correlation in cystic fibrosis: The role of modifier genes
    • Salvatore F, Scudiero O, Castaldo G. Genotype-phenotype correlation in cystic fibrosis: the role of modifier genes. Am J Med Genet 2002; 111: 88-95.
    • (2002) Am J Med Genet , vol.111 , pp. 88-95
    • Salvatore, F.1    Scudiero, O.2    Castaldo, G.3
  • 7
    • 0034109607 scopus 로고    scopus 로고
    • Genotype and phenotype in cystic fibrosis
    • Zielenski J. Genotype and phenotype in cystic fibrosis. Respiration 2000; 67: 117-133.
    • (2000) Respiration , vol.67 , pp. 117-133
    • Zielenski, J.1
  • 9
    • 0037081739 scopus 로고    scopus 로고
    • Temporal regulation of CFTR expression during ovine lung development: Implications for CF gene therapy
    • Broackes-Carter F C, Mouchel N, Gill D, Hyde S, Bassett J, Harris A. Temporal regulation of CFTR expression during ovine lung development: implications for CF gene therapy. Hum Mol Genet 2002; 11: 125-131.
    • (2002) Hum Mol Genet , vol.11 , pp. 125-131
    • Broackes-Carter, F.C.1    Mouchel, N.2    Gill, D.3    Hyde, S.4    Bassett, J.5    Harris, A.6
  • 10
    • 0033646566 scopus 로고    scopus 로고
    • Active intestinal chloride secretion in human carriers of cystic fibrosis mutations: An evaluation of the hypothesis that heterozygotes have subnormal active intestinal chloride secretion
    • Hogenauer C, Santa Ana C A, Porter J L et al. Active intestinal chloride secretion in human carriers of cystic fibrosis mutations: an evaluation of the hypothesis that heterozygotes have subnormal active intestinal chloride secretion. Am J Hum Genet 2000; 67: 1422-1427.
    • (2000) Am J Hum Genet , vol.67 , pp. 1422-1427
    • Hogenauer, C.1    Santa Ana, C.A.2    Porter, J.L.3
  • 11
    • 0027502580 scopus 로고
    • Genetic basis of variable exon 9 skipping in cystic fibrosis transmembrane conductance regulator mRNA
    • Chu C S, Trapnell B C, Curristin S, Cutting G R, Crystal R G. Genetic basis of variable exon 9 skipping in cystic fibrosis transmembrane conductance regulator mRNA. Nat Genet 1993; 3: 151-156.
    • (1993) Nat Genet , vol.3 , pp. 151-156
    • Chu, C.S.1    Trapnell, B.C.2    Curristin, S.3    Cutting, G.R.4    Crystal, R.G.5
  • 12
    • 0036892322 scopus 로고    scopus 로고
    • Genotype and phenotype correlations in patients with cystic fibrosis and pancreatitis
    • Durno C, Corey M, Zielenski J, Tullis E, Tsui L C, Durie P. Genotype and phenotype correlations in patients with cystic fibrosis and pancreatitis. Gastroenterology 2002; 123: 1857-1864.
    • (2002) Gastroenterology , vol.123 , pp. 1857-1864
    • Durno, C.1    Corey, M.2    Zielenski, J.3    Tullis, E.4    Tsui, L.C.5    Durie, P.6
  • 13
    • 0027517995 scopus 로고
    • Correlation between genotype and phenotype in patients with cystic fibrosis
    • The Cystic Fibrosis Genotype-Phenotype Consortium
    • Correlation between genotype and phenotype in patients with cystic fibrosis. The Cystic Fibrosis Genotype-Phenotype Consortium. N Engl J Med 1993; 329: 1308-1313.
    • (1993) N Engl J Med , vol.329 , pp. 1308-1313
  • 14
    • 0031678410 scopus 로고    scopus 로고
    • Contribution of genetic factors other than CFTR to disease severity in cystic fibrosis
    • Hull J, Thomson A H. Contribution of genetic factors other than CFTR to disease severity in cystic fibrosis. Thorax 1998; 53: 1018-1021.
    • (1998) Thorax , vol.53 , pp. 1018-1021
    • Hull, J.1    Thomson, A.H.2
  • 15
    • 0037613817 scopus 로고    scopus 로고
    • Multi-center study of gene modifiers for CF lung disease: Clinical phenotype of homozygous patients (deltaF/deltaF) with "mild" versus "severe" lung disease
    • Knowles M R, Konstan M W, Handler A et al. Multi-center study of gene modifiers for CF lung disease: clinical phenotype of homozygous patients (deltaF/deltaF) with "mild" versus "severe" lung disease. Pediatr Pulmonol 2002; 24 (Suppl.): 222.
    • (2002) Pediatr Pulmonol , vol.24 , Issue.SUPPL. , pp. 222
    • Knowles, M.R.1    Konstan, M.W.2    Handler, A.3
  • 16
    • 0025831754 scopus 로고
    • Cystic fibrosis and congenital absence of the vas deferens
    • Rigot J M, Lafitte J J, Dumur V et al. Cystic fibrosis and congenital absence of the vas deferens. N Engl J Med 1991; 325: 64-65.
    • (1991) N Engl J Med , vol.325 , pp. 64-65
    • Rigot, J.M.1    Lafitte, J.J.2    Dumur, V.3
  • 17
    • 0028958565 scopus 로고
    • Increased incidence of cystic fibrosis gene mutations in adults with disseminated bronchiectasis
    • Pignatti P F, Bombieri C, Marigo C, Benetazzo M, Luisetti M. Increased incidence of cystic fibrosis gene mutations in adults with disseminated bronchiectasis. Hum Mol Genet 1995; 4: 635-639.
    • (1995) Hum Mol Genet , vol.4 , pp. 635-639
    • Pignatti, P.F.1    Bombieri, C.2    Marigo, C.3    Benetazzo, M.4    Luisetti, M.5
  • 18
    • 0032480253 scopus 로고    scopus 로고
    • Mutations of the cystic fibrosis gene in patients with chronic pancreatitis
    • Sharer N, Schwarz M, Malone G et al. Mutations of the cystic fibrosis gene in patients with chronic pancreatitis. N Engl J Med 1998; 339: 645-652.
    • (1998) N Engl J Med , vol.339 , pp. 645-652
    • Sharer, N.1    Schwarz, M.2    Malone, G.3
  • 19
    • 0029080413 scopus 로고
    • Relative expression of the human epithelial Na+ channel subunits in normal and cystic fibrosis airways
    • Burch L H, Talbot C R, Knowles M R, Canessa C M, Rossier B C, Boucher R C. Relative expression of the human epithelial Na+ channel subunits in normal and cystic fibrosis airways. Am J Physiol 1995; 269: C511-C518.
    • (1995) Am J Physiol , vol.269
    • Burch, L.H.1    Talbot, C.R.2    Knowles, M.R.3    Canessa, C.M.4    Rossier, B.C.5    Boucher, R.C.6
  • 21
    • 0030909647 scopus 로고    scopus 로고
    • Early alterations in airway mucociliary clearance and inflammation of the lamina propria in CF mice
    • Zahm J M, Gaillard D, Dupuit F et al. Early alterations in airway mucociliary clearance and inflammation of the lamina propria in CF mice. Am J Physiol 1997; 272: C853-C859.
    • (1997) Am J Physiol , vol.272
    • Zahm, J.M.1    Gaillard, D.2    Dupuit, F.3
  • 22
    • 0034953901 scopus 로고    scopus 로고
    • A newly discovered human pneumovirus isolated from young children with respiratory tract disease
    • van den Hoogen B G, de Jong J C, Groen J et al. A newly discovered human pneumovirus isolated from young children with respiratory tract disease. Nat Med 2001; 7: 719-724.
    • (2001) Nat Med , vol.7 , pp. 719-724
    • Van den Hoogen, B.G.1    De Jong, J.C.2    Groen, J.3
  • 24
    • 0029870085 scopus 로고    scopus 로고
    • Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid
    • Smith J J, Travis S M, Greenberg E P, Welsh M J. Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid. Cell 1996; 85: 229-236.
    • (1996) Cell , vol.85 , pp. 229-236
    • Smith, J.J.1    Travis, S.M.2    Greenberg, E.P.3    Welsh, M.J.4
  • 25
    • 0031918382 scopus 로고    scopus 로고
    • Elemental content of airway surface liquid from infants with cystic fibrosis
    • Hull J, Skinner W, Robertson C, Phelan P. Elemental content of airway surface liquid from infants with cystic fibrosis. Am J Respir Crit Care Med 1998; 157: 10-14.
    • (1998) Am J Respir Crit Care Med , vol.157 , pp. 10-14
    • Hull, J.1    Skinner, W.2    Robertson, C.3    Phelan, P.4
  • 26
    • 0030814886 scopus 로고    scopus 로고
    • Ion composition of airway surface liquid of patients with cystic fibrosis as compared with normal and disease-control subjects
    • Knowles M R, Robinson J M, Wood R E et al. Ion composition of airway surface liquid of patients with cystic fibrosis as compared with normal and disease-control subjects. J Clin Invest 1997; 100: 2588-2595.
    • (1997) J Clin Invest , vol.100 , pp. 2588-2595
    • Knowles, M.R.1    Robinson, J.M.2    Wood, R.E.3
  • 27
    • 0034885663 scopus 로고    scopus 로고
    • The CF salt controversy: In vivo observations and therapeutic approaches
    • Tarran R, Grubb B R, Parsons D et al. The CF salt controversy: in vivo observations and therapeutic approaches. Mol Cell 2001; 8: 149-158.
    • (2001) Mol Cell , vol.8 , pp. 149-158
    • Tarran, R.1    Grubb, B.R.2    Parsons, D.3
  • 28
    • 0032433707 scopus 로고    scopus 로고
    • Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease
    • Matsui H, Grubb B R, Tarran R et al. Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease. Cell 1998; 95: 1005-1015.
    • (1998) Cell , vol.95 , pp. 1005-1015
    • Matsui, H.1    Grubb, B.R.2    Tarran, R.3
  • 29
    • 0036631526 scopus 로고    scopus 로고
    • Fecal elastase-I is superior to fecal chymotrypsin in the assessment of pancreatic involvement in cystic fibrosis
    • Walkowiak J, Herzig K H, Strzykala K, Przyslawski J, Krawczynski M. Fecal elastase-I is superior to fecal chymotrypsin in the assessment of pancreatic involvement in cystic fibrosis. Pediatrics 2002; 110: e7.
    • (2002) Pediatrics , vol.110
    • Walkowiak, J.1    Herzig, K.H.2    Strzykala, K.3    Przyslawski, J.4    Krawczynski, M.5
  • 30
    • 0036314580 scopus 로고    scopus 로고
    • Nitric oxide in chronic airway inflammation in children: Diagnostic use and pathophysiological significance
    • Narang I, Ersu R, Wilson N M, Bush A. Nitric oxide in chronic airway inflammation in children: diagnostic use and pathophysiological significance. Thorax 2002; 57: 586-589.
    • (2002) Thorax , vol.57 , pp. 586-589
    • Narang, I.1    Ersu, R.2    Wilson, N.M.3    Bush, A.4


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.