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Volumn 179, Issue 11, 2009, Pages 1022-1028

Airway delivery of low-dose miglustat normalizes nasal potential difference in F508del cystic fibrosis mice

Author keywords

Chloride transport; Cystic fibrosis; Miglustat; Nasal potential difference; Sodium transport

Indexed keywords

4,4' DIISOTHIOCYANATOSTILBENE 2,2' DISULFONIC ACID; AMILORIDE; CHLORIDE CHANNEL; FORSKOLIN; MIGLUSTAT; PLACEBO; TRANSMEMBRANE CONDUCTANCE REGULATOR;

EID: 66249120003     PISSN: 1073449X     EISSN: 15354970     Source Type: Journal    
DOI: 10.1164/rccm.200901-0049OC     Document Type: Article
Times cited : (42)

References (42)
  • 2
    • 33645130994 scopus 로고    scopus 로고
    • Cystic fibrosis since 1938
    • Davis PB. Cystic fibrosis since 1938. Am J Respir Crit Care Med 2006;173:475-482.
    • (2006) Am J Respir Crit Care Med , vol.173 , pp. 475-482
    • Davis, P.B.1
  • 3
    • 0030970422 scopus 로고    scopus 로고
    • Cystic fibrosis transmembrane conductance regulator inverts protein kinase A-mediated regulation of epithelial sodium channel single channel kinetics
    • DOI 10.1074/jbc.272.22.14037
    • Stutts MJ, Rossier BC, Boucher RC. Cystic fibrosis transmembrane conductance regulator inverts protein kinase A-mediated regulation of epithelial sodium channel single channel kinetics. J Biol Chem 1997;272:14037-14040. (Pubitemid 27232803)
    • (1997) Journal of Biological Chemistry , vol.272 , Issue.22 , pp. 14037-14040
    • Stutts, M.J.1    Rossier, B.C.2    Boucher, R.C.3
  • 5
    • 0025987020 scopus 로고
    • Phosphorylation of the R domain by cAMP-dependent protein kinase regulates the CFTR chloride channel
    • Cheng SH, Rich DP, Marshall J, Gregory RJ, Welsh MJ, Smith AE. Phosphorylation of the R domain by cAMP-dependent protein kinase regulates the CFTR chloride channel. Cell 1991;66:1027-1036. (Pubitemid 121001734)
    • (1991) Cell , vol.66 , Issue.5 , pp. 1027-1036
    • Cheng, S.H.1    Rich, D.P.2    Marshall, J.3    Gregory, R.J.4    Welsh, M.J.5    Smith, A.E.6
  • 7
    • 0028559511 scopus 로고
    • Conformational maturation of CFTR but not its mutant counterpart (delta F508) occurs in the endoplasmic reticulum and requires ATP
    • Lukacs GL, Mohamed A, Kartner N, Chang XB, Riordan JR, Grinstein S. Conformational maturation of CFTR but not its mutant counterpart (delta F508) occurs in the endoplasmic reticulum and requires ATP. EMBO J 1994;13:6076-6086.
    • (1994) EMBO J , vol.13 , pp. 6076-6086
    • Lukacs, G.L.1    Mohamed, A.2    Kartner, N.3    Chang, X.B.4    Riordan, J.R.5    Grinstein, S.6
  • 8
    • 34548286919 scopus 로고    scopus 로고
    • Detection of cystic fibrosis transmembrane conductance regulator activity in early-phase clinical trials
    • DOI 10.1513/pats.200703-043BR
    • Rowe SM, Accurso F, Clancy JP. Detection of cystic fibrosis transmembrane conductance regulator activity in early-phase clinical trials. Proc Am Thorac Soc 2007;4:387-398. (Pubitemid 47330876)
    • (2007) Proceedings of the American Thoracic Society , vol.4 , Issue.4 , pp. 387-398
    • Rowe, S.M.1    Accurso, F.2    Clancy, J.P.3
  • 13
    • 10744226382 scopus 로고    scopus 로고
    • The role of the iminosugar N-butyldeoxynojirimycin (miglustat) in the management of type I (non-neuronopathic) Gaucher disease: A position statement
    • Advisory Council to the European Working Group on Gaucher Disease
    • Cox TM, Aerts JM, Andria G, Beck M, Belmatoug N, Bembi B, Chertkoff R, Vom Dahl S, Elstein D, Erikson A, et al.; Advisory Council to the European Working Group on Gaucher Disease. The role of the iminosugar N-butyldeoxynojirimycin (miglustat) in the management of type I (non-neuronopathic) Gaucher disease: a position statement. J Inherit Metab Dis 2003;26:513-526.
    • (2003) J Inherit Metab Dis , vol.26 , pp. 513-526
    • Cox, T.M.1    Aerts, J.M.2    Andria, G.3    Beck, M.4    Belmatoug, N.5    Bembi, B.6    Chertkoff, R.7    Vom Dahl, S.8    Elstein, D.9    Erikson, A.10
  • 15
    • 27244456033 scopus 로고    scopus 로고
    • Imino sugar inhibitors for treating the lysosomal glycosphingolipidoses
    • DOI 10.1093/glycob/cwi076
    • Butters TD, Dwek RA, Platt FM. Imino sugar inhibitors for treating the lysosomal glycosphingolipidoses. Glycobiology 2005;15:43R-52R. (Pubitemid 41511483)
    • (2005) Glycobiology , vol.15 , Issue.10
    • Butters, T.D.1    Dwek, R.A.2    Platt, F.M.3
  • 16
    • 35648940330 scopus 로고    scopus 로고
    • Natural iminosugar derivatives of 1-deoxynojirimycin inhibit glycosylation of hepatitis viral envelope proteins
    • Jacob JR, Mansfiels K, You JR, Tennant BC, Kim YH. Natural iminosugar derivatives of 1-deoxynojirimycin inhibit glycosylation of hepatitis viral envelope proteins. J Microbiol 2007;45:431-440. (Pubitemid 350023378)
    • (2007) Journal of Microbiology , vol.45 , Issue.5 , pp. 431-440
    • Jacob, J.R.1    Mansfield, K.2    You, J.E.3    Tennant, B.C.4    Kim, Y.H.5
  • 17
    • 33645550893 scopus 로고    scopus 로고
    • Antiviral effect of alpha-glucosidase inhibitors on viral morphogenesis and binding properties of hepatitis C virus-like particles
    • Chapel C, Garcia C, Roingeard P, Zitzmann N, Dubuisson J, Dwek RA, Trépo C, Zoulim F, Durantel D. Antiviral effect of alpha-glucosidase inhibitors on viral morphogenesis and binding properties of hepatitis C virus-like particles. J Gen Virol 2006;87:861-871.
    • (2006) J Gen Virol , vol.87 , pp. 861-871
    • Chapel, C.1    Garcia, C.2    Roingeard, P.3    Zitzmann, N.4    Dubuisson, J.5    Dwek, R.A.6    Trépo, C.7    Zoulim, F.8    Durantel, D.9
  • 18
    • 33645211759 scopus 로고    scopus 로고
    • Rescue of functional delF508-CFTR channels in cystic fibrosis epithelial cells by the alpha-glucosidase inhibitor miglustat
    • Norez C, Noel S, Wilke M, Bijvelds M, Jorna H, Melin P, DeJonge H, Becq F. Rescue of functional delF508-CFTR channels in cystic fibrosis epithelial cells by the alpha-glucosidase inhibitor miglustat. FEBS Lett 2006;580:2081-2086.
    • (2006) FEBS Lett , vol.580 , pp. 2081-2086
    • Norez, C.1    Noel, S.2    Wilke, M.3    Bijvelds, M.4    Jorna, H.5    Melin, P.6    DeJonge, H.7    Becq, F.8
  • 19
    • 38149091749 scopus 로고    scopus 로고
    • Calcium homeostasis is abnormal in cystic fibrosis airway epithelial cells but is normalized after rescue of F508del-CFTR
    • Antigny F, Norez C, Becq F, Vandebrouck C. Calcium homeostasis is abnormal in cystic fibrosis airway epithelial cells but is normalized after rescue of F508del-CFTR. Cell Calcium 2008;43:175-183.
    • (2008) Cell Calcium , vol.43 , pp. 175-183
    • Antigny, F.1    Norez, C.2    Becq, F.3    Vandebrouck, C.4
  • 20
    • 44249097535 scopus 로고    scopus 로고
    • Parallel improvement of sodium and chloride transport defects by miglustat (n-butyldeoxynojyrimicin) in cystic fibrosis epithelial cells
    • Noël S, Wilke M, Bot AG, De Jonge HR, Becq F. Parallel improvement of sodium and chloride transport defects by miglustat (n-butyldeoxynojyrimicin) in cystic fibrosis epithelial cells. J Pharmacol Exp Ther 2008;325:1016-1023.
    • (2008) J Pharmacol Exp Ther , vol.325 , pp. 1016-1023
    • Noël, S.1    Wilke, M.2    Bot, A.G.3    De Jonge, H.R.4    Becq, F.5
  • 22
    • 85070846936 scopus 로고    scopus 로고
    • A CF respiratory cell chronically treated by miglustat acquires a non-CF like phenotype
    • Epub ahead of print
    • Norez C, Antigny F, Noel S, Vandenbrouck C, Becq F. A CF respiratory cell chronically treated by miglustat acquires a non-CF like phenotype. Am J Respir Cell Mol Biol 2009 Jan 8 [Epub ahead of print].
    • (2009) Am J Respir Cell Mol Biol , vol.8
    • Norez, C.1    Antigny, F.2    Noel, S.3    Vandenbrouck, C.4    Becq, F.5
  • 24
    • 0036145133 scopus 로고    scopus 로고
    • Recommendations for the health monitoring of rodent and rabbit colonies in breeding and experimental units
    • DOI 10.1258/0023677021911740
    • Nicklas W, Baneux P, Boot R, Decelle T, Deeny AA, Fumanelli M, Illgen-Wilcke B; FELASA (Federation of European Laboratory Animal Science Associations Working Group on Health Monitoring of Rodent and Rabbit Colonies). Recommendations for the health monitoring of rodent and rabbit colonies in breeding and experimental units. Lab Anim 2002;36:20-42. (Pubitemid 34073119)
    • (2002) Laboratory Animals , vol.36 , Issue.1 , pp. 20-42
    • Nicklas, W.1    Baneux, P.2    Boot, R.3    Decelle, T.4    Deeny, A.A.5    Fumanelli, M.6    Illgen-Wilcke, B.7
  • 26
    • 35048862316 scopus 로고    scopus 로고
    • The delivery site of a monovalent influenza vaccine within the respiratory tract impacts on the immune response
    • DOI 10.1111/j.1365-2567.2007.02641.x
    • Minne A, Louahed J, Mahauden S, Baras R, Renauld JC, Vanbever R. The delivery site of a monovalent influenza vaccine within the respiratory tract impacts on the immune response. Immunology 2007;122:316-325. (Pubitemid 47556468)
    • (2007) Immunology , vol.122 , Issue.3 , pp. 316-325
    • Minne, A.1    Louahed, J.2    Mehauden, S.3    Baras, B.4    Renauld, J.-C.5    Vanbever, R.6
  • 27
    • 0028171083 scopus 로고
    • N-Butyldeoxygalactonojirimycin inhibits glycolipid biosynthesis but does not affect N-linked oligosaccharide processing
    • Platt FM, Neises GR, Karlsson GB, Dwek RA, Butters TD. N-butyldeoxygalactonojirimycin inhibits glycolipid biosynthesis but does not affect N-linked oligosaccharide processing. J Biol Chem 1994;269:27108-27114. (Pubitemid 2150955)
    • (1994) Journal of Biological Chemistry , vol.269 , Issue.43 , pp. 27108-27114
    • Platt, F.M.1    Neises, G.R.2    Karlsson, G.B.3    Dwek, R.A.4    Butters, T.D.5
  • 28
    • 33644787306 scopus 로고    scopus 로고
    • Successful protocol of anaesthesia for measuring transepithelial nasal potential difference in spontaneously breathing mice
    • DOI 10.1258/002367706775404480
    • Leal T, Lebacq J, Vanbinst R, Lederman Ch, De Kock M, Wallemacq P. Successful protocol of anaesthesia for measuring transepithelial nasal potential difference in spontaneously breathing mice. Lab Anim 2006;40:43-52. (Pubitemid 43341082)
    • (2006) Laboratory Animals , vol.40 , Issue.1 , pp. 43-52
    • Leal, T.1    Lebacq, J.2    Vanbinst, R.3    Lederman, Ch.4    De Kock, M.5    Wallemacq, P.6
  • 29
    • 0028991826 scopus 로고
    • In vivo nasal potential difference: Techniques and protocols for assessing efficacy of gene transfer in cystic fibrosis
    • Knowles MR, Paradiso AM, Boucher RC. In vivo nasal potential difference: techniques and protocols for assessing efficacy of gene transfer in cystic fibrosis. Hum Gene Ther 1995;6:445-455.
    • (1995) Hum Gene Ther , vol.6 , pp. 445-455
    • Knowles, M.R.1    Paradiso, A.M.2    Boucher, R.C.3
  • 30
    • 0028020058 scopus 로고
    • Protocols for in vivo measurement of the ion transport defects in cystic fibrosis nasal epithelium
    • Middleton PG, Geddes DM, Alton EW. Protocols for in vivo measurement of the ion transport defects in cystic fibrosis nasal epithelium. Eur Respir J 1994;7:2050-2056. (Pubitemid 24366300)
    • (1994) European Respiratory Journal , vol.7 , Issue.11 , pp. 2050-2056
    • Middleton, P.G.1    Geddes, D.M.2    Alton, E.W.F.W.3
  • 34
    • 0038094385 scopus 로고    scopus 로고
    • Inhalation therapy: Technological milestones in asthma treatment
    • DOI 10.1016/S0169-409X(03)00077-2
    • Dalby R, Suman J. Inhalation therapy: technological milestones in asthma treatment. Adv Drug Deliv Rev 2003;55:779-791. (Pubitemid 36776793)
    • (2003) Advanced Drug Delivery Reviews , vol.55 , Issue.7 , pp. 779-791
    • Dalby, R.1    Suman, J.2
  • 35
    • 0026651508 scopus 로고
    • Chloride channels in the apical membrane of normal and cystic fibrosis airway and intestinal epithelia
    • Anderson MP, Sheppard DN, Berger HA, Welsh MJ. Chloride channels in the apical membrane of normal and cystic fibrosis airway and intestinal epithelia. Am J Physiol 1992;263:L1-L14.
    • (1992) Am J Physiol , vol.263
    • Anderson, M.P.1    Sheppard, D.N.2    Berger, H.A.3    Welsh, M.J.4
  • 37
    • 20344378216 scopus 로고    scopus 로고
    • Most F508del-CFTR is targeted to degradation at an early folding checkpoint and independently of calnexin
    • DOI 10.1128/MCB.25.12.5242-5252.2005
    • Farinha CM, Amaral MD. Most F508del-CFTR is targeted to degradation at an early folding checkpoint and independently of calnexin. Mol Cell Biol 2005;25:5242-5252. (Pubitemid 40781121)
    • (2005) Molecular and Cellular Biology , vol.25 , Issue.12 , pp. 5242-5252
    • Farinha, C.M.1    Amaral, M.D.2
  • 39
    • 58149279835 scopus 로고    scopus 로고
    • Assembly and misassembly of cystic fibrosis transmembrane conductance regulator: Folding defects caused by deletion of F508 occur before and after the calnexin-dependent association of membrane spanning domain (MSD) 1 and MSD2
    • Rosser MF, Grove DE, Chen L, Cyr DM. Assembly and misassembly of cystic fibrosis transmembrane conductance regulator: folding defects caused by deletion of F508 occur before and after the calnexin-dependent association of membrane spanning domain (MSD) 1 and MSD2. Mol Biol Cell 2008;19:4570-4579.
    • (2008) Mol Biol Cell , vol.19 , pp. 4570-4579
    • Rosser, M.F.1    Grove, D.E.2    Chen, L.3    Cyr, D.M.4
  • 40
    • 2442718786 scopus 로고    scopus 로고
    • + absorption produces cystic fibrosis-like lung disease in mice
    • DOI 10.1038/nm1028
    • Mall M, Grubb BR, Harkema JR, O'Neal WK, Boucher RC. Increased airway epithelial Na+ absorption produces cystic fibrosis-like lung disease in mice. Nat Med 2004;10:487-493. (Pubitemid 38667907)
    • (2004) Nature Medicine , vol.10 , Issue.5 , pp. 487-493
    • Mall, M.1    Grubb, B.R.2    Harkema, J.R.3    O'Neal, W.K.4    Boucher, R.C.5


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