메뉴 건너뛰기




Volumn 126, Issue 3, 2016, Pages 879-891

Acidic pH increases airway surface liquid viscosity in cystic fibrosis

Author keywords

[No Author keywords available]

Indexed keywords

CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; HEPARIN; MUCIN; SODIUM CHLORIDE; BICARBONATE; MESSENGER RNA; METHACHOLINE CHLORIDE; MUCIN 5AC; MUCIN 5B; POLYSACCHARIDE;

EID: 84959908589     PISSN: 00219738     EISSN: 15588238     Source Type: Journal    
DOI: 10.1172/JCI83922     Document Type: Article
Times cited : (201)

References (99)
  • 1
    • 0032913809 scopus 로고    scopus 로고
    • Physiological basis of cystic fibrosis: A historical perspective
    • Quinton P. Physiological basis of cystic fibrosis: a historical perspective. Physiol Rev. 1999;79(suppl 1):S3-S22.
    • (1999) Physiol Rev , vol.79 , pp. S3-S22
    • Quinton, P.1
  • 2
    • 84927176500 scopus 로고    scopus 로고
    • Cystic fibrosis genetics: From molecular understanDing to clinical application
    • Cutting GR. Cystic fibrosis genetics: from molecular understanDing to clinical application. Nat Rev Genet. 2015;16(1):45-56.
    • (2015) Nat Rev Genet , vol.16 , Issue.1 , pp. 45-56
    • Cutting, G.R.1
  • 3
    • 0029934215 scopus 로고    scopus 로고
    • Management of pulmonary disease in patients with cystic fibrosis
    • Ramsey BW. Management of pulmonary disease in patients with cystic fibrosis. N Engl J Med. 1996;335(3):179-188.
    • (1996) N Engl J Med , vol.335 , Issue.3 , pp. 179-188
    • Ramsey, B.W.1
  • 4
    • 52949154301 scopus 로고    scopus 로고
    • Disruption of the CFTR gene produces a model of cystic fibrosis in newborn pigs
    • Rogers CS, et al. Disruption of the CFTR gene produces a model of cystic fibrosis in newborn pigs. Science. 2008;321(5897):1837-1841.
    • (2008) Science , vol.321 , Issue.5897 , pp. 1837-1841
    • Rogers, C.S.1
  • 5
    • 79952779452 scopus 로고    scopus 로고
    • The F508 mutation causes CFTR misprocessing and cystic fibrosis-like disease in pigs
    • Ostedgaard LS, et al. The F508 mutation causes CFTR misprocessing and cystic fibrosis-like disease in pigs. Sci Transl Med. 2011;3(74):74ra24.
    • (2011) Sci Transl Med , vol.3 , Issue.74 , pp. 74ra24
    • Ostedgaard, L.S.1
  • 6
    • 77952974496 scopus 로고    scopus 로고
    • Cystic fibrosis pigs develop lung disease and exhibit defective bacterial eradication at birth
    • Stoltz DA, et al. Cystic fibrosis pigs develop lung disease and exhibit defective bacterial eradication at birth. Sci Transl Med. 2010;2(29):29ra31.
    • (2010) Sci Transl Med , vol.2 , Issue.29 , pp. 29ra31
    • Stoltz, D.A.1
  • 7
    • 84921455260 scopus 로고    scopus 로고
    • Origins of cystic fibrosis lung disease
    • Stoltz DA, Meyerholz DK, Welsh MJ. Origins of cystic fibrosis lung disease. N Engl J Med. 2015;372(4):351-362.
    • (2015) N Engl J Med , vol.372 , Issue.4 , pp. 351-362
    • Stoltz, D.A.1    Meyerholz, D.K.2    Welsh, M.J.3
  • 8
    • 84863476402 scopus 로고    scopus 로고
    • Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung
    • Pezzulo AA, et al. Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung. Nature. 2012;487(7405):109-113.
    • (2012) Nature , vol.487 , Issue.7405 , pp. 109-113
    • Pezzulo, A.A.1
  • 9
    • 84924565068 scopus 로고    scopus 로고
    • PH modulates the activity and synergism of the airway surface liquid antimicrobials defensin-3 and LL-37
    • Abou Alaiwa MH, et al. pH modulates the activity and synergism of the airway surface liquid antimicrobials defensin-3 and LL-37. Proc Natl Acad Sci U S A. 2014;111(52):18703-18708.
    • (2014) Proc Natl Acad Sci U S A , vol.111 , Issue.52 , pp. 18703-18708
    • Abou Alaiwa, M.H.1
  • 10
    • 84906089250 scopus 로고    scopus 로고
    • Impaired mucus detachment disrupts mucociliary transport in a piglet model of cystic fibrosis
    • Hoegger MJ, et al. Impaired mucus detachment disrupts mucociliary transport in a piglet model of cystic fibrosis. Science. 2014;345(698):818-822.
    • (2014) Science , vol.345 , Issue.698 , pp. 818-822
    • Hoegger, M.J.1
  • 11
    • 84865643337 scopus 로고    scopus 로고
    • Sinus hypoplasia precedes sinus infection in a porcine model of cystic fibrosis
    • Chang EH, et al. Sinus hypoplasia precedes sinus infection in a porcine model of cystic fibrosis. Laryngoscope. 2012;122(9):1898-1905.
    • (2012) Laryngoscope , vol.122 , Issue.9 , pp. 1898-1905
    • Chang, E.H.1
  • 13
    • 0036208055 scopus 로고    scopus 로고
    • Mucociliary clearance in cystic fibrosis
    • Robinson M, Bye PT. Mucociliary clearance in cystic fibrosis. Pediatr Pulmonol. 2002;33(4):293-306.
    • (2002) Pediatr Pulmonol , vol.33 , Issue.4 , pp. 293-306
    • Robinson, M.1    Bye, P.T.2
  • 14
    • 4544353992 scopus 로고    scopus 로고
    • Submucosal glands and airway defense
    • Wine JJ, Joo NS. Submucosal glands and airway defense. Proc Am Thorac Soc. 2004;1(1):47-53.
    • (2004) Proc Am Thorac Soc , vol.1 , Issue.1 , pp. 47-53
    • Wine, J.J.1    Joo, N.S.2
  • 15
    • 78649735324 scopus 로고    scopus 로고
    • Airway mucus function and dysfunction
    • Fahy JV, Dickey BF. Airway mucus function and dysfunction. N Engl J Med. 2010;363(23):2233-2247.
    • (2010) N Engl J Med , vol.363 , Issue.23 , pp. 2233-2247
    • Fahy, J.V.1    Dickey, B.F.2
  • 16
    • 0024990329 scopus 로고
    • The role of mucous glycoproteins in the rheologic properties of cystic fibrosis sputum
    • Lethem MI, James SL, Marriott C. The role of mucous glycoproteins in the rheologic properties of cystic fibrosis sputum. Am Rev Respir Dis. 1990;142(5):1053-1058.
    • (1990) Am Rev Respir Dis , vol.142 , Issue.5 , pp. 1053-1058
    • Lethem, M.I.1    James, S.L.2    Marriott, C.3
  • 18
    • 84973088441 scopus 로고    scopus 로고
    • Secretion properties, clearance, and therapy in airway disease
    • Rubin BK. Secretion properties, clearance, and therapy in airway disease. Transl Respir Med. 2014;2:6.
    • (2014) Transl Respir Med , vol.2 , pp. 6
    • Rubin, B.K.1
  • 19
    • 84907833958 scopus 로고    scopus 로고
    • A functional anatomic defect of the cystic fibrosis airway
    • Birket SE, et al. A functional anatomic defect of the cystic fibrosis airway. Am J Respir Crit Care Med. 2014;190(4):421-432.
    • (2014) Am J Respir Crit Care Med , vol.190 , Issue.4 , pp. 421-432
    • Birket, S.E.1
  • 20
    • 43549093774 scopus 로고    scopus 로고
    • Structure and function of the polymeric mucins in airways mucus
    • Thornton DJ, Rousseau K, McGuckin MA. Structure and function of the polymeric mucins in airways mucus. Annu Rev Physiol. 2008;70:459-486.
    • (2008) Annu Rev Physiol , vol.70 , pp. 459-486
    • Thornton, D.J.1    Rousseau, K.2    McGuckin, M.A.3
  • 21
    • 33745001604 scopus 로고    scopus 로고
    • Mucin structure, aggregation, physiological functions and biomedical applications
    • Bansil R, Turner BS. Mucin structure, aggregation, physiological functions and biomedical applications. Curr Opin Colloid Interface Sci. 2006;11(2-3):164-170.
    • (2006) Curr Opin Colloid Interface Sci , vol.11 , Issue.2-3 , pp. 164-170
    • Bansil, R.1    Turner, B.S.2
  • 24
    • 0036499905 scopus 로고    scopus 로고
    • Concentrated solutions of salivary MUC5B mucin do not replicate the gel-forming properties of saliva
    • Raynal BD, Hardingham TE, Thornton DJ, Sheehan JK. Concentrated solutions of salivary MUC5B mucin do not replicate the gel-forming properties of saliva. Biochem J. 2002;362(pt 2):289-296.
    • (2002) Biochem J , vol.362 , pp. 289-296
    • Raynal, B.D.1    Hardingham, T.E.2    Thornton, D.J.3    Sheehan, J.K.4
  • 25
    • 25144450137 scopus 로고    scopus 로고
    • Altered O-glycosylation and sulfation of airway mucins associated with cystic fibrosis
    • Xia B, Royall JA, Damera G, Sachdev GP, Cummings RD. Altered O-glycosylation and sulfation of airway mucins associated with cystic fibrosis. Glycobiology. 2005;15(8):747-775.
    • (2005) Glycobiology , vol.15 , Issue.8 , pp. 747-775
    • Xia, B.1    Royall, J.A.2    Damera, G.3    Sachdev, G.P.4    Cummings, R.D.5
  • 26
    • 21044432997 scopus 로고    scopus 로고
    • Mucin glycosylation changes in cystic fibrosis lung disease are not manifest in submucosal gland secretions
    • Schulz BL, et al. Mucin glycosylation changes in cystic fibrosis lung disease are not manifest in submucosal gland secretions. Biochem J. 2005;387(pt 3):911-919.
    • (2005) Biochem J , vol.387 , pp. 911-919
    • Schulz, B.L.1
  • 27
    • 34447345699 scopus 로고    scopus 로고
    • Glycosylation of sputum mucins is altered in cystic fibrosis patients
    • Schulz BL, et al. Glycosylation of sputum mucins is altered in cystic fibrosis patients. Glycobiology. 2007;17(7):698-712.
    • (2007) Glycobiology , vol.17 , Issue.7 , pp. 698-712
    • Schulz, B.L.1
  • 28
    • 0035189941 scopus 로고    scopus 로고
    • Altered sialyl-and fucosyl-linkage on mucins in cystic fibrosis patients promotes formation of the sialyl-Lewis X determinant on salivary MUC-5B and MUC-7
    • Shori DK, et al. Altered sialyl-and fucosyl-linkage on mucins in cystic fibrosis patients promotes formation of the sialyl-Lewis X determinant on salivary MUC-5B and MUC-7. Pflugers Arch. 2001;443(suppl 1):S55-S61.
    • (2001) Pflugers Arch , vol.443 , pp. S55-S61
    • Shori, D.K.1
  • 29
    • 0031735960 scopus 로고    scopus 로고
    • Different O-glycosylation of respiratory mucin glycopeptides from a patient with cystic fibrosis
    • Thomsson KA, Carlstedt I, Karlsson NG, Karlsson H, Hansson GC. Different O-glycosylation of respiratory mucin glycopeptides from a patient with cystic fibrosis. Glycoconj J. 1998;15(8):823-833.
    • (1998) Glycoconj J , vol.15 , Issue.8 , pp. 823-833
    • Thomsson, K.A.1    Carlstedt, I.2    Karlsson, N.G.3    Karlsson, H.4    Hansson, G.C.5
  • 30
    • 78349237748 scopus 로고    scopus 로고
    • Loss of CFTR function produces abnormalities in tracheal development in neonatal pigs and young children
    • Meyerholz DK, et al. Loss of CFTR function produces abnormalities in tracheal development in neonatal pigs and young children. Am J Respir Crit Care Med. 2010;182(10):1251-1261.
    • (2010) Am J Respir Crit Care Med , vol.182 , Issue.10 , pp. 1251-1261
    • Meyerholz, D.K.1
  • 31
    • 79960003498 scopus 로고    scopus 로고
    • Hyperviscous airway periciliary and mucous liquid layers in cystic fibrosis measured by confocal fluorescence photobleaching
    • Derichs N, Jin BJ, Song Y, Finkbeiner WE, Verkman AS. Hyperviscous airway periciliary and mucous liquid layers in cystic fibrosis measured by confocal fluorescence photobleaching. FASEB J. 2011;25(7):2325-2332.
    • (2011) FASEB J , vol.25 , Issue.7 , pp. 2325-2332
    • Derichs, N.1    Jin, B.J.2    Song, Y.3    Finkbeiner, W.E.4    Verkman, A.S.5
  • 33
    • 84892549076 scopus 로고    scopus 로고
    • Distinguishing free and anomalous diffusion by rectangular fluorescence recovery after photobleaching: A Monte Carlo study
    • De Clercq B, Cleuren B, Deschout H, Braeckmans K, Ameloot M. Distinguishing free and anomalous diffusion by rectangular fluorescence recovery after photobleaching: a Monte Carlo study. J Biomed Opt. 2013;18(7):76012.
    • (2013) J Biomed Opt , vol.18 , Issue.7 , pp. 76012
    • De Clercq, B.1    Cleuren, B.2    Deschout, H.3    Braeckmans, K.4    Ameloot, M.5
  • 34
    • 0031913234 scopus 로고    scopus 로고
    • Inhibition of airway liquid secretion and its effect on the physical properties of airway mucus
    • Trout L, King M, Feng W, Inglis SK, Ballard ST. Inhibition of airway liquid secretion and its effect on the physical properties of airway mucus. Am J Physiol Cell Physiol. 1998;274(2 pt 1):L258-L263.
    • (1998) Am J Physiol Cell Physiol , vol.274 , Issue.2 , pp. L258-L263
    • Trout, L.1    King, M.2    Feng, W.3    Inglis, S.K.4    Ballard, S.T.5
  • 35
    • 3843101723 scopus 로고    scopus 로고
    • A small molecule CFTR inhibitor produces cystic fibrosis-like submucosal gland fluid secretions in normal airways
    • Thiagarajah JR, Song Y, Haggie PM, Verkman AS. A small molecule CFTR inhibitor produces cystic fibrosis-like submucosal gland fluid secretions in normal airways. FASEB J. 2004;18(7):875-877.
    • (2004) FASEB J , vol.18 , Issue.7 , pp. 875-877
    • Thiagarajah, J.R.1    Song, Y.2    Haggie, P.M.3    Verkman, A.S.4
  • 36
    • 79961089536 scopus 로고    scopus 로고
    • Mucous solids and liquid secretion by airways: Studies with normal pig, cystic fibrosis human, and non-cystic fibrosis human bronchi
    • Martens CJ, et al. Mucous solids and liquid secretion by airways: studies with normal pig, cystic fibrosis human, and non-cystic fibrosis human bronchi. Am J Physiol Lung Cell Mol Physiol. 2011;301(2):L236-L246.
    • (2011) Am J Physiol Lung Cell Mol Physiol , vol.301 , Issue.2 , pp. L236-L246
    • Martens, C.J.1
  • 37
    • 78649915334 scopus 로고    scopus 로고
    • Loss of anion transport without increased sodium absorption characterize new-born porcine cystic fibrosis airway epithelia
    • Chen J-H, et al. Loss of anion transport without increased sodium absorption characterize new-born porcine cystic fibrosis airway epithelia. Cell. 2010;143(6):911-923.
    • (2010) Cell , vol.143 , Issue.6 , pp. 911-923
    • Chen, J.-H.1
  • 38
    • 77956249987 scopus 로고    scopus 로고
    • Airway surface liquid depth imaged by surface laser reflectance microscopy
    • Thiagarajah JR, Song Y, Derichs N, Verkman AS. Airway surface liquid depth imaged by surface laser reflectance microscopy. J Gen Physiol. 2010;136(3):353-362.
    • (2010) J Gen Physiol , vol.136 , Issue.3 , pp. 353-362
    • Thiagarajah, J.R.1    Song, Y.2    Derichs, N.3    Verkman, A.S.4
  • 39
    • 79952216879 scopus 로고    scopus 로고
    • Quantification of periciliary fluid (PCL) height in human airway biopsies is feasible, but not suitable as a biomarker
    • Griesenbach U, et al. Quantification of periciliary fluid (PCL) height in human airway biopsies is feasible, but not suitable as a biomarker. Am J Respir Cell Mol Biol. 2010;44(3):309-315.
    • (2010) Am J Respir Cell Mol Biol , vol.44 , Issue.3 , pp. 309-315
    • Griesenbach, U.1
  • 40
    • 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, et al. Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease. Cell. 1998;95(7):1005-1015.
    • (1998) Cell , vol.95 , Issue.7 , pp. 1005-1015
    • Matsui, H.1
  • 41
    • 84923870560 scopus 로고    scopus 로고
    • Oxidation increases mucin polymer cross-links to stiffen airway mucus gels
    • Yuan S, et al. Oxidation increases mucin polymer cross-links to stiffen airway mucus gels. Sci Transl Med. 2015;7(276):276ra227.
    • (2015) Sci Transl Med , vol.7 , Issue.276 , pp. 276ra227
    • Yuan, S.1
  • 42
    • 33644620356 scopus 로고    scopus 로고
    • Respiratory tract mucin genes and mucin glycoproteins in health and disease
    • Rose MC, Voynow JA. Respiratory tract mucin genes and mucin glycoproteins in health and disease. Physiol Rev. 2006;86(1):245-278.
    • (2006) Physiol Rev , vol.86 , Issue.1 , pp. 245-278
    • Rose, M.C.1    Voynow, J.A.2
  • 43
    • 0029016145 scopus 로고
    • Alteration of sulfation of glycoconjugates, but not sulfate transport and intracellular inorganic sulfate content in cystic fibrosis airway epithelial cells
    • Mohapatra NK, et al. Alteration of sulfation of glycoconjugates, but not sulfate transport and intracellular inorganic sulfate content in cystic fibrosis airway epithelial cells. Pediatr Res. 1995;38(1):42-48.
    • (1995) Pediatr Res , vol.38 , Issue.1 , pp. 42-48
    • Mohapatra, N.K.1
  • 44
    • 0032869010 scopus 로고    scopus 로고
    • Terminal glycosylation in cystic fibrosis
    • Scanlin TF, Glick MC. Terminal glycosylation in cystic fibrosis. Biochim Biophys Acta. 1999;1455(2-3):241-253.
    • (1999) Biochim Biophys Acta , vol.1455 , Issue.2-3 , pp. 241-253
    • Scanlin, T.F.1    Glick, M.C.2
  • 45
    • 0028804840 scopus 로고
    • Genotypic analysis of respiratory mucous sulfation defects in cystic fibrosis
    • Zhang Y, Doranz B, Yankaskas JR, Engelhardt JF. Genotypic analysis of respiratory mucous sulfation defects in cystic fibrosis. J Clin Invest. 1995;96(6):2997-3004.
    • (1995) J Clin Invest , vol.96 , Issue.6 , pp. 2997-3004
    • Zhang, Y.1    Doranz, B.2    Yankaskas, J.R.3    Engelhardt, J.F.4
  • 46
    • 77952261296 scopus 로고    scopus 로고
    • Characterization of particle translocation through mucin hydrogels
    • Lieleg O, Vladescu I, Ribbeck K. Characterization of particle translocation through mucin hydrogels. Biophys J. 2010;98(9):1782-1789.
    • (2010) Biophys J , vol.98 , Issue.9 , pp. 1782-1789
    • Lieleg, O.1    Vladescu, I.2    Ribbeck, K.3
  • 47
    • 0026321416 scopus 로고
    • Profound increase in viscosity and aggregation of pig gastric mucin at low pH
    • Bhaskar KR, et al. Profound increase in viscosity and aggregation of pig gastric mucin at low pH. Am J Physiol Cell Physiol. 1991;261(5 pt 1):G827-G832.
    • (1991) Am J Physiol Cell Physiol , vol.261 , Issue.5 , pp. G827-G832
    • Bhaskar, K.R.1
  • 48
    • 0024400646 scopus 로고
    • Subunit interactions and physical properties of bovine gallbladder mucin
    • Smith BF, Peetermans JA, Tanaka T, LaMont JT. Subunit interactions and physical properties of bovine gallbladder mucin. Gastroenterology. 1989;97(1):179-187.
    • (1989) Gastroenterology , vol.97 , Issue.1 , pp. 179-187
    • Smith, B.F.1    Peetermans, J.A.2    Tanaka, T.3    LaMont, J.T.4
  • 49
    • 0024781055 scopus 로고
    • Viscosity of human salivary mucins: Effect of pH and ionic strength and role of sialic acid
    • Veerman EC, Valentijn-Benz M, Nieuw Amerongen AV. Viscosity of human salivary mucins: effect of pH and ionic strength and role of sialic acid. J Biol Buccale. 1989;17(4):297-306.
    • (1989) J Biol Buccale , vol.17 , Issue.4 , pp. 297-306
    • Veerman, E.C.1    Valentijn-Benz, M.2    Nieuw Amerongen, A.V.3
  • 50
    • 48149090717 scopus 로고    scopus 로고
    • Cystic fibrosis: Impaired bicarbonate secretion and mucoviscidosis
    • Quinton PM. Cystic fibrosis: impaired bicarbonate secretion and mucoviscidosis. Lancet. 2008;372(9636):415-417.
    • (2008) Lancet , vol.372 , Issue.9636 , pp. 415-417
    • Quinton, P.M.1
  • 52
    • 70349223856 scopus 로고    scopus 로고
    • Normal mouse intestinal mucus release requires cystic fibrosis transmembrane regulator-dependent bicarbonate secretion
    • Garcia MA, Yang N, Quinton PM. Normal mouse intestinal mucus release requires cystic fibrosis transmembrane regulator-dependent bicarbonate secretion. J Clin Invest. 2009;119(9):2613-2622.
    • (2009) J Clin Invest , vol.119 , Issue.9 , pp. 2613-2622
    • Garcia, M.A.1    Yang, N.2    Quinton, P.M.3
  • 53
    • 84864313785 scopus 로고    scopus 로고
    • Bicarbonate and functional CFTR channel are required for proper mucin secretion and link cystic fibrosis with its mucus phenotype
    • Gustafsson JK, et al. Bicarbonate and functional CFTR channel are required for proper mucin secretion and link cystic fibrosis with its mucus phenotype. J Exp Med. 2012;209(7):1263-1272.
    • (2012) J Exp Med , vol.209 , Issue.7 , pp. 1263-1272
    • Gustafsson, J.K.1
  • 54
    • 77954464369 scopus 로고    scopus 로고
    • A new role for bicarbonate secretion in cervico-uterine mucus release
    • Muchekehu RW, Quinton PM. A new role for bicarbonate secretion in cervico-uterine mucus release. J Physiol. 2010;588(pt 13):2329-2342.
    • (2010) J Physiol , vol.588 , pp. 2329-2342
    • Muchekehu, R.W.1    Quinton, P.M.2
  • 55
    • 0346103648 scopus 로고    scopus 로고
    • Abnormal surface liquid pH regulation by cultured cystic fibrosis bronchial epithelium
    • Coakley RD, et al. Abnormal surface liquid pH regulation by cultured cystic fibrosis bronchial epithelium. Proc Natl Acad Sci U S A. 2003;100(26):16083-16088.
    • (2003) Proc Natl Acad Sci U S A , vol.100 , Issue.26 , pp. 16083-16088
    • Coakley, R.D.1
  • 56
    • 84904343311 scopus 로고    scopus 로고
    • The buffer capacity of airway epithelial secretions
    • Kim D, Liao J, Hanrahan JW. The buffer capacity of airway epithelial secretions. Front Physiol. 2014;5:188.
    • (2014) Front Physiol , vol.5 , pp. 188
    • Kim, D.1    Liao, J.2    Hanrahan, J.W.3
  • 57
    • 25444529982 scopus 로고    scopus 로고
    • Anionic poly(amino acid)s dissolve F-actin and DNA bundles, enhance DNase activity, and reduce the viscosity of cystic fibrosis sputum
    • Tang JX, et al. Anionic poly(amino acid)s dissolve F-actin and DNA bundles, enhance DNase activity, and reduce the viscosity of cystic fibrosis sputum. Am J Physiol Lung Cell Mol Physiol. 2005;289(4):L599-L605.
    • (2005) Am J Physiol Lung Cell Mol Physiol , vol.289 , Issue.4 , pp. L599-L605
    • Tang, J.X.1
  • 59
    • 0041434124 scopus 로고
    • Molecular mechanism of product storage and release in mucin secretion. II. The role of extracellular Ca++
    • Verdugo P, Aitken M, Langley L, Villalon MJ. Molecular mechanism of product storage and release in mucin secretion. II. The role of extracellular Ca++. Biorheology. 1987;24(6):625-633.
    • (1987) Biorheology , vol.24 , Issue.6 , pp. 625-633
    • Verdugo, P.1    Aitken, M.2    Langley, L.3    Villalon, M.J.4
  • 60
    • 0025306375 scopus 로고
    • Goblet cells secretion and mucogenesis
    • Verdugo P. Goblet cells secretion and mucogenesis. Annu Rev Physiol. 1990;52:157-176.
    • (1990) Annu Rev Physiol , vol.52 , pp. 157-176
    • Verdugo, P.1
  • 61
    • 0042206801 scopus 로고    scopus 로고
    • Calcium-dependent protein interactions in MUC5B provide reversible cross-links in salivary mucus
    • Raynal BD, Hardingham TE, Sheehan JK, Thornton DJ. Calcium-dependent protein interactions in MUC5B provide reversible cross-links in salivary mucus. J Biol Chem. 2003;278(31):28703-28710.
    • (2003) J Biol Chem , vol.278 , Issue.31 , pp. 28703-28710
    • Raynal, B.D.1    Hardingham, T.E.2    Sheehan, J.K.3    Thornton, D.J.4
  • 62
    • 0027141851 scopus 로고
    • Elemental composition of human airway surface fluid in healthy and diseased airways
    • Joris L, Dab I, Quinton PM. Elemental composition of human airway surface fluid in healthy and diseased airways. Am Rev Respir Dis. 1993;148(6 pt 1):1633-1637.
    • (1993) Am Rev Respir Dis , vol.148 , Issue.6 , pp. 1633-1637
    • Joris, L.1    Dab, I.2    Quinton, P.M.3
  • 64
    • 33645007857 scopus 로고    scopus 로고
    • X-ray microanalysis of apical fluid in cystic fibrosis airway epithelial cell lines
    • Kozlova I, Nilsson H, Henriksnas J, Roomans GM. X-ray microanalysis of apical fluid in cystic fibrosis airway epithelial cell lines. Cell Physiol Biochem. 2006;17(1-2):13-20.
    • (2006) Cell Physiol Biochem , vol.17 , Issue.1-2 , pp. 13-20
    • Kozlova, I.1    Nilsson, H.2    Henriksnas, J.3    Roomans, G.M.4
  • 66
    • 84902150768 scopus 로고    scopus 로고
    • Assembly of the respiratory mucin MUC5B: A new model for a gel-forming mucin
    • Ridley C, et al. Assembly of the respiratory mucin MUC5B: a new model for a gel-forming mucin. J Biol Chem. 2014;289(23):16409-16420.
    • (2014) J Biol Chem , vol.289 , Issue.23 , pp. 16409-16420
    • Ridley, C.1
  • 67
    • 0035988475 scopus 로고    scopus 로고
    • Acidic pH and increasing [Ca(2+)] reduce the swelling of mucins in primary cultures of human cervical cells
    • Espinosa M, Noe G, Troncoso C, Ho SB, Villalon M. Acidic pH and increasing [Ca(2+)] reduce the swelling of mucins in primary cultures of human cervical cells. Hum Reprod. 2002;17(8):1964-1972.
    • (2002) Hum Reprod , vol.17 , Issue.8 , pp. 1964-1972
    • Espinosa, M.1    Noe, G.2    Troncoso, C.3    Ho, S.B.4    Villalon, M.5
  • 68
    • 34249870086 scopus 로고    scopus 로고
    • Rheology of gastric mucin exhibits a pH-dependent sol-gel transition
    • Celli JP, et al. Rheology of gastric mucin exhibits a pH-dependent sol-gel transition. Biomacromolecules. 2007;8(5):1580-1586.
    • (2007) Biomacromolecules , vol.8 , Issue.5 , pp. 1580-1586
    • Celli, J.P.1
  • 69
    • 0022021791 scopus 로고
    • Influence of buffer capacity and pH-dependent rheological properties of respiratory mucus on health effects due to acidic pollution
    • Holma B. Influence of buffer capacity and pH-dependent rheological properties of respiratory mucus on health effects due to acidic pollution. Sci Total Environ. 1985;41(2):101-123.
    • (1985) Sci Total Environ , vol.41 , Issue.2 , pp. 101-123
    • Holma, B.1
  • 71
    • 4544354750 scopus 로고    scopus 로고
    • Mucins and their O-glycans from human bronchial epithelial cell cultures
    • Holmen JM, et al. Mucins and their O-glycans from human bronchial epithelial cell cultures. Am J Physiol Lung Cell Mol Physiol. 2004;287(4):L824-L834.
    • (2004) Am J Physiol Lung Cell Mol Physiol , vol.287 , Issue.4 , pp. L824-L834
    • Holmen, J.M.1
  • 72
    • 0032983991 scopus 로고    scopus 로고
    • The sialylation of bronchial mucins secreted by patients suffering from cystic fibrosis or from chronic bronchitis is related to the severity of airway infection
    • Davril M, et al. The sialylation of bronchial mucins secreted by patients suffering from cystic fibrosis or from chronic bronchitis is related to the severity of airway infection. Glycobiology. 1999;9(3):311-321.
    • (1999) Glycobiology , vol.9 , Issue.3 , pp. 311-321
    • Davril, M.1
  • 73
    • 0028133685 scopus 로고
    • Heterologous expression of F508 CFTR results in decreased sialylation of membrane glycoconjugates
    • Dosanjh A, Lencer W, Brown D, Ausiello DA, Stow JL. Heterologous expression of F508 CFTR results in decreased sialylation of membrane glycoconjugates. Am J Physiol. 1994; 266(2 pt 1):C360-C366.
    • (1994) Am J Physiol , vol.266 , Issue.2 , pp. C360-C366
    • Dosanjh, A.1    Lencer, W.2    Brown, D.3    Ausiello, D.A.4    Stow, J.L.5
  • 74
    • 0025784535 scopus 로고
    • Defective acidification of intracellular organelles in cystic fibrosis
    • Barasch J, et al. Defective acidification of intracellular organelles in cystic fibrosis. Nature. 1991;352(6330):70-73.
    • (1991) Nature , vol.352 , Issue.6330 , pp. 70-73
    • Barasch, J.1
  • 75
    • 0033696130 scopus 로고    scopus 로고
    • Evidence against the acidification hypothesis in cystic fibrosis
    • Gibson GA, Hill WG, Weisz OA. Evidence against the acidification hypothesis in cystic fibrosis. Am J Physiol Cell Physiol. 2000;279(4):C1088-C1099.
    • (2000) Am J Physiol Cell Physiol , vol.279 , Issue.4 , pp. C1088-C1099
    • Gibson, G.A.1    Hill, W.G.2    Weisz, O.A.3
  • 76
    • 2442454780 scopus 로고    scopus 로고
    • Identification of molecular intermediates in the assembly pathway of the MUC5AC mucin
    • Sheehan JK, et al. Identification of molecular intermediates in the assembly pathway of the MUC5AC mucin. J Biol Chem. 2004;279(15):15698-15705.
    • (2004) J Biol Chem , vol.279 , Issue.15 , pp. 15698-15705
    • Sheehan, J.K.1
  • 77
    • 84867893683 scopus 로고    scopus 로고
    • FolDing of pig gastric mucin non-glycosylated domains: A discrete molecular dynamics study
    • Barz B, Turner BS, Bansil R, Urbanc B. FolDing of pig gastric mucin non-glycosylated domains: a discrete molecular dynamics study. J Biol Phys. 2012;38(4):681-703.
    • (2012) J Biol Phys , vol.38 , Issue.4 , pp. 681-703
    • Barz, B.1    Turner, B.S.2    Bansil, R.3    Urbanc, B.4
  • 78
    • 84857932056 scopus 로고    scopus 로고
    • Calcium stabilizes the von Willebrand factor A2 domain by promoting refolding
    • Xu AJ, Springer TA. Calcium stabilizes the von Willebrand factor A2 domain by promoting refolding. Proc Natl Acad Sci U S A. 2012;109(10):3742-3747.
    • (2012) Proc Natl Acad Sci U S A , vol.109 , Issue.10 , pp. 3742-3747
    • Xu, A.J.1    Springer, T.A.2
  • 79
    • 0016654205 scopus 로고
    • Calcium binDing to intestinal goblet cell mucin
    • Forstner JF, Forstner GG. Calcium binDing to intestinal goblet cell mucin. Biochim Biophys Acta. 1975;386(1):283-292.
    • (1975) Biochim Biophys Acta , vol.386 , Issue.1 , pp. 283-292
    • Forstner, J.F.1    Forstner, G.G.2
  • 80
    • 84902333159 scopus 로고    scopus 로고
    • Neonates with cystic fibrosis have a reduced nasal liquid pH: A small pilot study
    • Abou Alaiwa MH, et al. Neonates with cystic fibrosis have a reduced nasal liquid pH: A small pilot study. J Cyst Fibros. 2014;13(4):373-377.
    • (2014) J Cyst Fibros , vol.13 , Issue.4 , pp. 373-377
    • Abou Alaiwa, M.H.1
  • 81
    • 0037252641 scopus 로고    scopus 로고
    • Airway surface pH in subjects with cystic fibrosis
    • McShane D, et al. Airway surface pH in subjects with cystic fibrosis. Eur Respir J. 2003;21(1):37-42.
    • (2003) Eur Respir J , vol.21 , Issue.1 , pp. 37-42
    • McShane, D.1
  • 82
    • 84885084821 scopus 로고    scopus 로고
    • Molecular basis for pH-dependent mucosal dehydration in cystic fibrosis airways
    • Garland AL, et al. Molecular basis for pH-dependent mucosal dehydration in cystic fibrosis airways. Proc Natl Acad Sci U S A. 2013;110(40):15973-15978.
    • (2013) Proc Natl Acad Sci U S A , vol.110 , Issue.40 , pp. 15973-15978
    • Garland, A.L.1
  • 83
    • 0034949080 scopus 로고    scopus 로고
    • Nebulized heparin in Burkholderia cepacia colonized adult cystic fibrosis patients
    • Ledson M, Gallagher M, Hart CA, Walshaw M. Nebulized heparin in Burkholderia cepacia colonized adult cystic fibrosis patients. Eur Respir J. 2001;17(1):36-38.
    • (2001) Eur Respir J , vol.17 , Issue.1 , pp. 36-38
    • Ledson, M.1    Gallagher, M.2    Hart, C.A.3    Walshaw, M.4
  • 84
    • 30944466084 scopus 로고    scopus 로고
    • A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis
    • Elkins MR, et al. A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis. N Engl J Med. 2006;354(3):229-240.
    • (2006) N Engl J Med , vol.354 , Issue.3 , pp. 229-240
    • Elkins, M.R.1
  • 85
    • 30944452384 scopus 로고    scopus 로고
    • Mucus clearance and lung function in cystic fibrosis with hypertonic saline
    • Donaldson SH, et al. Mucus clearance and lung function in cystic fibrosis with hypertonic saline. N Engl J Med. 2006;354(3):241-250.
    • (2006) N Engl J Med , vol.354 , Issue.3 , pp. 241-250
    • Donaldson, S.H.1
  • 86
    • 0021130061 scopus 로고
    • Cation induced changes in the rheological properties of purified mucus glycoprotein gels
    • Crowther RS, Marriott C, James SL. Cation induced changes in the rheological properties of purified mucus glycoprotein gels. Biorheology. 1984;21(1-2):253-263.
    • (1984) Biorheology , vol.21 , Issue.1-2 , pp. 253-263
    • Crowther, R.S.1    Marriott, C.2    James, S.L.3
  • 87
    • 0024367288 scopus 로고
    • Physical properties of purified human respiratory mucus glycoproteins: Effects of sodium chloride concentration on the aggregation properties and shape
    • Chace KV, Naziruddin B, Desai VC, Flux M, Sachdev GP. Physical properties of purified human respiratory mucus glycoproteins: effects of sodium chloride concentration on the aggregation properties and shape. Exp Lung Res. 1989;15(5):721-737.
    • (1989) Exp Lung Res , vol.15 , Issue.5 , pp. 721-737
    • Chace, K.V.1    Naziruddin, B.2    Desai, V.C.3    Flux, M.4    Sachdev, G.P.5
  • 88
    • 84919665852 scopus 로고    scopus 로고
    • Hypertonic saline releases the attached small intestinal cystic fibrosis mucus
    • Ermund A, Meiss LN, Scholte BJ, Hansson GC. Hypertonic saline releases the attached small intestinal cystic fibrosis mucus. Clin Exp Pharmacol Physiol. 2015;42(1):69-75.
    • (2015) Clin Exp Pharmacol Physiol , vol.42 , Issue.1 , pp. 69-75
    • Ermund, A.1    Meiss, L.N.2    Scholte, B.J.3    Hansson, G.C.4
  • 89
    • 0032484910 scopus 로고    scopus 로고
    • Spontaneous assembly of marine dissolved organic matter into polymer gels
    • Chin W-C, Orellana MV, Verdugo P. Spontaneous assembly of marine dissolved organic matter into polymer gels. Nature. 1998;391:568-572.
    • (1998) Nature , vol.391 , pp. 568-572
    • Chin, W.-C.1    Orellana, M.V.2    Verdugo, P.3
  • 90
    • 0021840423 scopus 로고
    • Edetate sodium aerosol in Pseudomonas lung infection in cystic fibrosis
    • Brown J, Mellis CM, Wood RE. Edetate sodium aerosol in Pseudomonas lung infection in cystic fibrosis. Am J Dis Child. 1985;139(8):836-839.
    • (1985) Am J Dis Child , vol.139 , Issue.8 , pp. 836-839
    • Brown, J.1    Mellis, C.M.2    Wood, R.E.3
  • 92
    • 0037092556 scopus 로고    scopus 로고
    • PH in expired breath condensate of patients with inflammatory airway diseases
    • Kostikas K, et al. pH in expired breath condensate of patients with inflammatory airway diseases. Am J Respir Crit Care Med. 2002;165(10):1364-1370.
    • (2002) Am J Respir Crit Care Med , vol.165 , Issue.10 , pp. 1364-1370
    • Kostikas, K.1
  • 93
    • 0242319772 scopus 로고    scopus 로고
    • Exhaled breath condensate acidification in acute lung injury
    • Gessner C, et al. Exhaled breath condensate acidification in acute lung injury. Respir Med. 2003;97(11):1188-1194.
    • (2003) Respir Med , vol.97 , Issue.11 , pp. 1188-1194
    • Gessner, C.1
  • 94
    • 0036367311 scopus 로고    scopus 로고
    • An in vitro model of differentiated human airway epithelia: Methods and evaluation of primary cultures
    • In: Wise C, ed.. Totowa, New Jersey, USA: Humana Press, Inc
    • Karp PH, et al. An in vitro model of differentiated human airway epithelia: methods and evaluation of primary cultures. In: Wise C, ed. Epithelial Cell Culture Protocols. Vol. 188. Totowa, New Jersey, USA: Humana Press, Inc.; 2002:115-137.
    • (2002) Epithelial Cell Culture Protocols , vol.188 , pp. 115-137
    • Karp, P.H.1
  • 95
    • 77956354814 scopus 로고    scopus 로고
    • Hyposecretion of fluid from tracheal submucosal glands of CFTR-deficient pigs
    • Joo NS, Cho HJ, Khansaheb M, Wine JJ. Hyposecretion of fluid from tracheal submucosal glands of CFTR-deficient pigs. J Clin Invest. 2010;120(9):3161-3166.
    • (2010) J Clin Invest , vol.120 , Issue.9 , pp. 3161-3166
    • Joo, N.S.1    Cho, H.J.2    Khansaheb, M.3    Wine, J.J.4
  • 96
    • 0014408452 scopus 로고
    • Structures and immunochemical properties of oligosaccharides isolated from pig submaxillary mucins
    • Carlson DM. Structures and immunochemical properties of oligosaccharides isolated from pig submaxillary mucins. J Biol Chem. 1968;243(3):616-626.
    • (1968) J Biol Chem , vol.243 , Issue.3 , pp. 616-626
    • Carlson, D.M.1
  • 97
    • 66149149459 scopus 로고    scopus 로고
    • Analysis of N-and O-linked glycans from glycoproteins using MALDI-TOF mass spectrometry
    • Morelle W, Faid V, Chirat F, Michalski JC. Analysis of N-and O-linked glycans from glycoproteins using MALDI-TOF mass spectrometry. Methods Mol Biol. 2009;534:5-21.
    • (2009) Methods Mol Biol , vol.534 , pp. 5-21
    • Morelle, W.1    Faid, V.2    Chirat, F.3    Michalski, J.C.4
  • 98
    • 0023877998 scopus 로고
    • Monosaccharide analysis of glycoconjugates by anion exchange chromatography with pulsed amperometric detection
    • Hardy MR, Townsend RR, Lee YC. Monosaccharide analysis of glycoconjugates by anion exchange chromatography with pulsed amperometric detection. Anal Biochem. 1988;170(1):54-62.
    • (1988) Anal Biochem , vol.170 , Issue.1 , pp. 54-62
    • Hardy, M.R.1    Townsend, R.R.2    Lee, Y.C.3
  • 99
    • 3343011972 scopus 로고    scopus 로고
    • Discovery and characterization of sialic acid O-acetylation in group B Streptococcus
    • Lewis AL, Nizet V, Varki A. Discovery and characterization of sialic acid O-acetylation in group B Streptococcus. Proc Natl Acad Sci U S A. 2004;101(30):11123-11128.
    • (2004) Proc Natl Acad Sci U S A , vol.101 , Issue.30 , pp. 11123-11128
    • Lewis, A.L.1    Nizet, V.2    Varki, A.3


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.