메뉴 건너뛰기




Volumn 3, Issue 3, 2006, Pages 251-259

Cellular and animal models of cystic fibrosis, tools for drug discovery

Author keywords

[No Author keywords available]

Indexed keywords

AMINOGLYCOSIDE ANTIBIOTIC AGENT; ANTIINFLAMMATORY AGENT; PHENYLGLYCINE; PHOSPHODIESTERASE INHIBITOR; PYRAZINE DERIVATIVE; PYRAZOLE DERIVATIVE; SMALL INTERFERING RNA; SULFONAMIDE; TETRAHYDROTHIOPHENE DERIVATIVE; TRANSMEMBRANE CONDUCTANCE REGULATOR;

EID: 33751239440     PISSN: 17406757     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.ddmod.2006.09.003     Document Type: Review
Times cited : (9)

References (46)
  • 1
    • 33645307384 scopus 로고    scopus 로고
    • The ABC protein turned chloride channel whose failure causes cystic fibrosis
    • Gadsby D.C., et al. The ABC protein turned chloride channel whose failure causes cystic fibrosis. Nature 440 (2006) 477-483
    • (2006) Nature , vol.440 , pp. 477-483
    • Gadsby, D.C.1
  • 2
    • 15544371839 scopus 로고    scopus 로고
    • Assembly of functional CFTR chloride channels
    • Riordan J.R. Assembly of functional CFTR chloride channels. Annu. Rev. Physiol. 67 (2005) 701-718
    • (2005) Annu. Rev. Physiol. , vol.67 , pp. 701-718
    • Riordan, J.R.1
  • 3
    • 33744957047 scopus 로고    scopus 로고
    • Cysteine string protein monitors late steps in cystic fibrosis transmembrane conductance regulator biogenesis
    • Zhang H., et al. Cysteine string protein monitors late steps in cystic fibrosis transmembrane conductance regulator biogenesis. J. Biol. Chem. 281 (2006) 11312-11321
    • (2006) J. Biol. Chem. , vol.281 , pp. 11312-11321
    • Zhang, H.1
  • 4
    • 27744482654 scopus 로고    scopus 로고
    • The short apical membrane half-life of rescued {Delta}F508-cystic fibrosis transmembrane conductance regulator (CFTR) results from accelerated endocytosis of {Delta}F508-CFTR in polarized human airway epithelial cells
    • Swiatecka-Urban A., et al. The short apical membrane half-life of rescued {Delta}F508-cystic fibrosis transmembrane conductance regulator (CFTR) results from accelerated endocytosis of {Delta}F508-CFTR in polarized human airway epithelial cells. J. Biol. Chem. 280 (2005) 36762-36772
    • (2005) J. Biol. Chem. , vol.280 , pp. 36762-36772
    • Swiatecka-Urban, A.1
  • 5
    • 33745772850 scopus 로고    scopus 로고
    • New insights into cystic fibrosis: molecular switches that regulate CFTR
    • Guggino W.B., and Stanton B.A. New insights into cystic fibrosis: molecular switches that regulate CFTR. Nat. Rev. Mol. Cell Biol. 7 (2006) 426-436
    • (2006) Nat. Rev. Mol. Cell Biol. , vol.7 , pp. 426-436
    • Guggino, W.B.1    Stanton, B.A.2
  • 6
    • 0034109607 scopus 로고    scopus 로고
    • Genotype and phenotype in cystic fibrosis
    • Zielenski J. Genotype and phenotype in cystic fibrosis. Respiration 67 (2000) 117-133
    • (2000) Respiration , vol.67 , pp. 117-133
    • Zielenski, J.1
  • 7
    • 0141615605 scopus 로고    scopus 로고
    • The phenotypic consequences of CFTR mutations
    • Rowntree R.K., and Harris A. The phenotypic consequences of CFTR mutations. Ann. Hum. Genet. 67 PT 5 (2003) 471-485
    • (2003) Ann. Hum. Genet. , vol.67 , Issue.PART 5 , pp. 471-485
    • Rowntree, R.K.1    Harris, A.2
  • 8
    • 17444400836 scopus 로고    scopus 로고
    • Modifier genes in cystic fibrosis
    • Davies J.C., et al. Modifier genes in cystic fibrosis. Pediatr. Pulmonol. 39 (2005) 383-391
    • (2005) Pediatr. Pulmonol. , vol.39 , pp. 383-391
    • Davies, J.C.1
  • 10
    • 0026532895 scopus 로고
    • Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR)
    • Bear C.E., et al. Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR). Cell 68 (1992) 809-818
    • (1992) Cell , vol.68 , pp. 809-818
    • Bear, C.E.1
  • 11
    • 18944361982 scopus 로고    scopus 로고
    • CFTR fails to inhibit the epithelial sodium channel ENaC expressed in Xenopus laevis oocytes
    • Nagel G., et al. CFTR fails to inhibit the epithelial sodium channel ENaC expressed in Xenopus laevis oocytes. J. Physiol. 564 Pt 3 (2005) 671-682
    • (2005) J. Physiol. , vol.564 , Issue.PART 3 , pp. 671-682
    • Nagel, G.1
  • 12
    • 0030176455 scopus 로고    scopus 로고
    • Chloride channels: a molecular perspective
    • Jentsch T.J. Chloride channels: a molecular perspective. Curr. Opin. Neurobiol. 6 (1996) 303-310
    • (1996) Curr. Opin. Neurobiol. , vol.6 , pp. 303-310
    • Jentsch, T.J.1
  • 13
    • 11044227865 scopus 로고    scopus 로고
    • Established cell lines used in cystic fibrosis research
    • Gruenert D.C., et al. Established cell lines used in cystic fibrosis research. J. Cyst. Fibros. 3 Suppl. 2 (2004) 191-196
    • (2004) J. Cyst. Fibros. , vol.3 , Issue.SUPPL. 2 , pp. 191-196
    • Gruenert, D.C.1
  • 14
    • 11044229344 scopus 로고    scopus 로고
    • Isolation and air-liquid interface culture of human large airway and bronchiolar epithelial cells
    • Bals R., et al. Isolation and air-liquid interface culture of human large airway and bronchiolar epithelial cells. J. Cyst. Fibros. 3 S2 (2004) 49-51
    • (2004) J. Cyst. Fibros. 3 , vol.S2 , pp. 49-51
    • Bals, R.1
  • 15
    • 1442325340 scopus 로고    scopus 로고
    • Cytokine secretion by cystic fibrosis airway epithelial cells
    • Becker M.N., et al. Cytokine secretion by cystic fibrosis airway epithelial cells. Am. J. Respir. Crit. Care Med. 169 (2004) 645-653
    • (2004) Am. J. Respir. Crit. Care Med. , vol.169 , pp. 645-653
    • Becker, M.N.1
  • 16
    • 0033679720 scopus 로고    scopus 로고
    • A primary culture model of differentiated murine tracheal epithelium [In Process Citation]
    • Davidson D.J., et al. A primary culture model of differentiated murine tracheal epithelium [In Process Citation]. Am. J. Physiol. Lung Cell Mol. Physiol. 279 (2000) L766-L778
    • (2000) Am. J. Physiol. Lung Cell Mol. Physiol. , vol.279
    • Davidson, D.J.1
  • 17
    • 33644836771 scopus 로고    scopus 로고
    • Culture of murine nasal epithelia: model for cystic fibrosis
    • Grubb B.R., et al. Culture of murine nasal epithelia: model for cystic fibrosis. Am. J. Physiol. Lung Cell Mol. Physiol. 290 (2006) L270-L277
    • (2006) Am. J. Physiol. Lung Cell Mol. Physiol. , vol.290
    • Grubb, B.R.1
  • 18
    • 0036012527 scopus 로고    scopus 로고
    • Xenograft model of the CF airway
    • Filali M., et al. Xenograft model of the CF airway. Methods Mol. Med. 70 (2002) 537-550
    • (2002) Methods Mol. Med. , vol.70 , pp. 537-550
    • Filali, M.1
  • 19
    • 0032775601 scopus 로고    scopus 로고
    • Ion transport across the normal and CF neonatal murine intestine
    • Grubb B.R. Ion transport across the normal and CF neonatal murine intestine. Am. J. Physiol. 277 1 Pt 1 (1999) G167-G174
    • (1999) Am. J. Physiol. , vol.277 , Issue.1 PART 1
    • Grubb, B.R.1
  • 20
    • 11044232030 scopus 로고    scopus 로고
    • Ex vivo CF diagnosis by intestinal current measurements (ICM) in small aperture, circulating Ussing chambers
    • De Jonge H.R., et al. Ex vivo CF diagnosis by intestinal current measurements (ICM) in small aperture, circulating Ussing chambers. J. Cyst. Fibros. 3 Suppl. 2 (2004) 159-163
    • (2004) J. Cyst. Fibros. , vol.3 , Issue.SUPPL. 2 , pp. 159-163
    • De Jonge, H.R.1
  • 21
    • 0028218460 scopus 로고
    • Determinants of mild clinical symptoms in cystic fibrosis patients. Residual chloride secretion measured in rectal biopsies in relation to the genotype
    • Veeze H.J., et al. Determinants of mild clinical symptoms in cystic fibrosis patients. Residual chloride secretion measured in rectal biopsies in relation to the genotype. J. Clin. Invest. 93 (1994) 461-466
    • (1994) J. Clin. Invest. , vol.93 , pp. 461-466
    • Veeze, H.J.1
  • 22
    • 85012499363 scopus 로고    scopus 로고
    • The CF mouse: an important tool for studying cystic fibrosis
    • (http://www-ermm.cbcu.cam.ac.uk/01002551h.htm)
    • Davidson D.J., and Dorin J.R. The CF mouse: an important tool for studying cystic fibrosis. Expert Rev. Mol. Med. (2001). http://www-ermm.cbcu.cam.ac.uk/01002551h.htm (http://www-ermm.cbcu.cam.ac.uk/01002551h.htm)
    • (2001) Expert Rev. Mol. Med.
    • Davidson, D.J.1    Dorin, J.R.2
  • 23
    • 11044233752 scopus 로고    scopus 로고
    • Animal models of cystic fibrosis
    • Scholte B.J., et al. Animal models of cystic fibrosis. J. Cyst. Fibros. 3 Suppl. 2 (2004) 183-190
    • (2004) J. Cyst. Fibros. , vol.3 , Issue.SUPPL. 2 , pp. 183-190
    • Scholte, B.J.1
  • 24
    • 0032962320 scopus 로고    scopus 로고
    • Pathophysiology of gene-targeted mouse models for cystic fibrosis
    • Grubb B.R., and Boucher R.C. Pathophysiology of gene-targeted mouse models for cystic fibrosis. Physiol. Rev. 79 Suppl. 1 (1999) S193-S214
    • (1999) Physiol. Rev. , vol.79 , Issue.SUPPL. 1
    • Grubb, B.R.1    Boucher, R.C.2
  • 25
    • 0028557095 scopus 로고
    • Correction of lethal intestinal defect in a mouse model of cystic fibrosis by human CFTR
    • Zhou L., et al. Correction of lethal intestinal defect in a mouse model of cystic fibrosis by human CFTR. Science 266 (1994) 1705-1708
    • (1994) Science , vol.266 , pp. 1705-1708
    • Zhou, L.1
  • 26
    • 32144455216 scopus 로고    scopus 로고
    • Response to acute lung infection with mucoid Pseudomonas aeruginosa in cystic fibrosis mice
    • van Heeckeren A.M., et al. Response to acute lung infection with mucoid Pseudomonas aeruginosa in cystic fibrosis mice. Am. J. Respir. Crit. Care Med. 173 (2006) 288-296
    • (2006) Am. J. Respir. Crit. Care Med. , vol.173 , pp. 288-296
    • van Heeckeren, A.M.1
  • 27
    • 0041701380 scopus 로고    scopus 로고
    • Pseudomonas aeruginosa-induced apoptosis is defective in respiratory epithelial cells expressing mutant cystic fibrosis transmembrane conductance regulator
    • Cannon C.L., et al. Pseudomonas aeruginosa-induced apoptosis is defective in respiratory epithelial cells expressing mutant cystic fibrosis transmembrane conductance regulator. Am. J. Respir. Cell Mol. Biol. 29 (2003) 188-197
    • (2003) Am. J. Respir. Cell Mol. Biol. , vol.29 , pp. 188-197
    • Cannon, C.L.1
  • 28
    • 2442718786 scopus 로고    scopus 로고
    • + absorption produces cystic fibrosis-like lung disease in mice
    • + absorption produces cystic fibrosis-like lung disease in mice. Nat. Med. 10 (2004) 487-493
    • (2004) Nat. Med. , vol.10 , pp. 487-493
    • Mall, M.1
  • 29
    • 33745755929 scopus 로고    scopus 로고
    • Gene therapy progress and prospects: cystic fibrosis
    • Griesenbach U., et al. Gene therapy progress and prospects: cystic fibrosis. Gene Ther. 13 (2006) 1061-1067
    • (2006) Gene Ther. , vol.13 , pp. 1061-1067
    • Griesenbach, U.1
  • 30
    • 33745454174 scopus 로고    scopus 로고
    • Gene and cell therapy for cystic fibrosis
    • Davies J.C. Gene and cell therapy for cystic fibrosis. Paediatr. Respir. Rev. 7 Suppl. 1 (2006) S163-S165
    • (2006) Paediatr. Respir. Rev. , vol.7 , Issue.SUPPL. 1
    • Davies, J.C.1
  • 31
    • 0242363119 scopus 로고    scopus 로고
    • Pharmacological approaches to correcting the ion transport defect in cystic fibrosis
    • Roomans G.M. Pharmacological approaches to correcting the ion transport defect in cystic fibrosis. Am. J. Respir. Med. 2 (2003) 413-431
    • (2003) Am. J. Respir. Med. , vol.2 , pp. 413-431
    • Roomans, G.M.1
  • 32
    • 0141863491 scopus 로고    scopus 로고
    • Gentamicin-induced correction of CFTR function in patients with cystic fibrosis and CFTR stop mutations
    • Wilschanski M., et al. Gentamicin-induced correction of CFTR function in patients with cystic fibrosis and CFTR stop mutations. N. Engl. J. Med. 349 (2003) 1433-1441
    • (2003) N. Engl. J. Med. , vol.349 , pp. 1433-1441
    • Wilschanski, M.1
  • 33
    • 24644464284 scopus 로고    scopus 로고
    • Small-molecule correctors of defective DeltaF508-CFTR cellular processing identified by high-throughput screening
    • Pedemonte N., et al. Small-molecule correctors of defective DeltaF508-CFTR cellular processing identified by high-throughput screening. J. Clin. Invest. 115 (2005) 2564-2571
    • (2005) J. Clin. Invest. , vol.115 , pp. 2564-2571
    • Pedemonte, N.1
  • 34
    • 33748987102 scopus 로고    scopus 로고
    • Discovery of pyrrolo[2,3-b]pyrazines derivatives as submicromolar affinity activators of wild-type, G551D and F508del CFTR chloride channels
    • Noel S., et al. Discovery of pyrrolo[2,3-b]pyrazines derivatives as submicromolar affinity activators of wild-type, G551D and F508del CFTR chloride channels. J. Pharmacol. Exp. Ther. 319 (2006) 349-359
    • (2006) J. Pharmacol. Exp. Ther. , vol.319 , pp. 349-359
    • Noel, S.1
  • 35
    • 33744831154 scopus 로고    scopus 로고
    • Rescue of DeltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules
    • Van Goor F., et al. Rescue of DeltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules. Am. J. Physiol. Lung Cell Mol. Physiol. 290 (2006) L1117-L1130
    • (2006) Am. J. Physiol. Lung Cell Mol. Physiol. , vol.290
    • Van Goor, F.1
  • 36
    • 33748944330 scopus 로고    scopus 로고
    • S-nitrosylating agents, a novel class of compounds that increase CFTR expression and maturation in epithelial cells
    • Zaman K., et al. S-nitrosylating agents, a novel class of compounds that increase CFTR expression and maturation in epithelial cells. Mol. Pharmacol. 70 (2006) 1435-1442
    • (2006) Mol. Pharmacol. , vol.70 , pp. 1435-1442
    • Zaman, K.1
  • 37
    • 1542675960 scopus 로고    scopus 로고
    • Cystic fibrosis: premature degradation of mutant proteins as a molecular disease mechanism
    • Gelman M.S., and Kopito R.R. Cystic fibrosis: premature degradation of mutant proteins as a molecular disease mechanism. Methods Mol. Biol. 232 (2003) 27-37
    • (2003) Methods Mol. Biol. , vol.232 , pp. 27-37
    • Gelman, M.S.1    Kopito, R.R.2
  • 38
    • 33745190973 scopus 로고    scopus 로고
    • Selective inhibition of endoplasmic reticulum-associated degradation rescues DeltaF508-cystic fibrosis transmembrane regulator and suppresses interleukin-8 levels: therapeutic implications
    • Vij N., et al. Selective inhibition of endoplasmic reticulum-associated degradation rescues DeltaF508-cystic fibrosis transmembrane regulator and suppresses interleukin-8 levels: therapeutic implications. J. Biol. Chem. 281 (2006) 17369-17378
    • (2006) J. Biol. Chem. , vol.281 , pp. 17369-17378
    • Vij, N.1
  • 39
    • 0346727128 scopus 로고    scopus 로고
    • Therapeutic approaches to protein-misfolding diseases
    • Cohen F.E., and Kelly J.W. Therapeutic approaches to protein-misfolding diseases. Nature 426 (2003) 905-909
    • (2003) Nature , vol.426 , pp. 905-909
    • Cohen, F.E.1    Kelly, J.W.2
  • 40
    • 33745121896 scopus 로고    scopus 로고
    • Burying the dead: the impact of failed apoptotic cell removal (efferocytosis) on chronic inflammatory lung disease
    • Vandivier R.W., et al. Burying the dead: the impact of failed apoptotic cell removal (efferocytosis) on chronic inflammatory lung disease. Chest 129 (2006) 1673-1682
    • (2006) Chest , vol.129 , pp. 1673-1682
    • Vandivier, R.W.1
  • 41
  • 42
    • 23644439540 scopus 로고    scopus 로고
    • Eradication of early Pseudomonas aeruginosa infection
    • Hoiby N., et al. Eradication of early Pseudomonas aeruginosa infection. J. Cyst. Fibros. 4 Suppl. 2 (2005) 49-54
    • (2005) J. Cyst. Fibros. , vol.4 , Issue.SUPPL. 2 , pp. 49-54
    • Hoiby, N.1
  • 43
    • 27544434744 scopus 로고    scopus 로고
    • Developmental paradigm for early features of cystic fibrosis
    • Larson J.E., and Cohen J.C. Developmental paradigm for early features of cystic fibrosis. Pediatr. Pulmonol. 40 (2005) 371-377
    • (2005) Pediatr. Pulmonol. , vol.40 , pp. 371-377
    • Larson, J.E.1    Cohen, J.C.2
  • 44
    • 1642277855 scopus 로고    scopus 로고
    • Characteristic multiorgan pathology of cystic fibrosis in a long-living cystic fibrosis transmembrane regulator knockout murine model
    • Durie P.R., et al. Characteristic multiorgan pathology of cystic fibrosis in a long-living cystic fibrosis transmembrane regulator knockout murine model. Am. J. Pathol. 164 (2004) 1481-1493
    • (2004) Am. J. Pathol. , vol.164 , pp. 1481-1493
    • Durie, P.R.1
  • 45
    • 19944414292 scopus 로고    scopus 로고
    • The patch-clamp and planar lipid bilayer techniques: powerful and versatile tools to investigate the CFTR Cl- channel
    • Sheppard D.N., et al. The patch-clamp and planar lipid bilayer techniques: powerful and versatile tools to investigate the CFTR Cl- channel. J. Cyst. Fibros. 3 Suppl. 2 (2004) 101-108
    • (2004) J. Cyst. Fibros. , vol.3 , Issue.SUPPL. 2 , pp. 101-108
    • Sheppard, D.N.1
  • 46
    • 33748146503 scopus 로고    scopus 로고
    • CFTR regulates phagosome acidification in macrophages and alters bactericidal activity
    • Di A., et al. CFTR regulates phagosome acidification in macrophages and alters bactericidal activity. Nat. Cell Biol. 8 9 (2006) 933-944
    • (2006) Nat. Cell Biol. , vol.8 , Issue.9 , pp. 933-944
    • Di, A.1


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.