메뉴 건너뛰기




Volumn 124, Issue 7, 2014, Pages 3047-3060

Cystic fibrosis airway secretions exhibit mucin hyperconcentration and increased osmotic pressure

Author keywords

[No Author keywords available]

Indexed keywords

LACTOFERRIN; LEUKOCYTE ELASTASE; MESSENGER RNA; MUCIN; MUCIN 5AC; MUCIN 5B;

EID: 84903759350     PISSN: 00219738     EISSN: 15588238     Source Type: Journal    
DOI: 10.1172/JCI73469     Document Type: Article
Times cited : (253)

References (49)
  • 1
    • 33645130994 scopus 로고    scopus 로고
    • Cystic fibrosis since 1938
    • Davis PB. Cystic fibrosis since 1938. Am J Respir Crit Care Med. 2006;173(5):475-482.
    • (2006) Am J Respir Crit Care Med , vol.173 , Issue.5 , pp. 475-482
    • Davis, P.B.1
  • 2
    • 84865305392 scopus 로고    scopus 로고
    • A periciliary brush promotes the lung health by separating the mucus layer from airway epithelia
    • Button B, et al. A periciliary brush promotes the lung health by separating the mucus layer from airway epithelia. Science. 2012;337(6097):937- 941.
    • (2012) Science , vol.337 , Issue.6097 , pp. 937-941
    • Button, B.1
  • 3
    • 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
  • 4
    • 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
    • Stoltz, D.A.1
  • 5
    • 84879410271 scopus 로고    scopus 로고
    • Distribution of early structural lung changes due to cystic fibrosis detected with chest computed tomography
    • Mott LS, et al. Distribution of early structural lung changes due to cystic fibrosis detected with chest computed tomography. J Pediatr. 2013; 163(1):243-248 e243.
    • (2013) J Pediatr , vol.163 , Issue.1 , pp. 243-248
    • Mott, L.S.1
  • 6
    • 0029870085 scopus 로고    scopus 로고
    • Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid
    • Smith JJ, Travis SM, Greenberg EP, Welsh MJ. Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid. Cell. 1996;85(2):229-236.
    • (1996) Cell , vol.85 , Issue.2 , pp. 229-236
    • Smith, J.J.1    Travis, S.M.2    Greenberg, E.P.3    Welsh, M.J.4
  • 7
    • 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
  • 8
    • 0036198121 scopus 로고    scopus 로고
    • Antimicrobial polypeptides in host defense of the respiratory tract
    • Ganz T. Antimicrobial polypeptides in host defense of the respiratory tract. J Clin Invest. 2002; 109(6):693-697.
    • (2002) J Clin Invest , vol.109 , Issue.6 , pp. 693-697
    • Ganz, T.1
  • 10
    • 0023491997 scopus 로고
    • Biochemical properties of tracheobronchial mucins from cystic fibrosis and non-cystic fibrosis individuals
    • Rose MC, Brown CF, Jacoby JZ, Jacoby JZ 3rd, Lynn WS, Kaufman B. Biochemical properties of tracheobronchial mucins from cystic fibrosis and non-cystic fibrosis individuals. Pediatr Res. 1987; 22(5):545-551.
    • (1987) Pediatr Res , vol.22 , Issue.5 , pp. 545-551
    • Rose, M.C.1    Brown, C.F.2    Jacoby, J.Z.3    Jacoby III, J.Z.4    Lynn, W.S.5    Kaufman, B.6
  • 12
    • 0029841922 scopus 로고    scopus 로고
    • Different mucins are produced by the surface epithelium and the submucosa in human trachea: Identification of MUC5AC as a major mucin from the goblet cells
    • Hovenberg HW, Davies JR, Carlstedt I. Different mucins are produced by the surface epithelium and the submucosa in human trachea: identification of MUC5AC as a major mucin from the goblet cells. Biochem J. 1996;318(pt 1):319-324.
    • (1996) Biochem J , vol.318 , Issue.PART 1 , pp. 319-324
    • Hovenberg, H.W.1    Davies, J.R.2    Carlstedt, I.3
  • 13
    • 0033406611 scopus 로고    scopus 로고
    • Identification of MUC5B, MUC5AC and small amounts of MUC2 mucins in cystic fibrosis airway secretions
    • Davies JR, Svitacheva N, Lannefors L, Kornfalt R, Carlstedt I. Identification of MUC5B, MUC5AC and small amounts of MUC2 mucins in cystic fibrosis airway secretions. Biochem J. 1999;344(pt 2):321-330.
    • (1999) Biochem J , vol.344 , Issue.PART 2 , pp. 321-330
    • Davies, J.R.1    Svitacheva, N.2    Lannefors, L.3    Kornfalt, R.4    Carlstedt, I.5
  • 14
    • 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
  • 15
    • 33847084360 scopus 로고    scopus 로고
    • A morphometric study of mucins and small airway plugging in cystic fibrosis
    • Burgel PR, Montani D, Danel C, Dusser DJ, Nadel JA. A morphometric study of mucins and small airway plugging in cystic fibrosis. Thorax. 2007;62(2):153-161.
    • (2007) Thorax , vol.62 , Issue.2 , pp. 153-161
    • Burgel, P.R.1    Montani, D.2    Danel, C.3    Dusser, D.J.4    Nadel, J.A.5
  • 16
    • 34047180932 scopus 로고    scopus 로고
    • MUC5AC and MUC5B mucins increase in cystic fibrosis airway secretions during pulmonary exacerbation
    • Henke MO, John G, Germann M, Lindemann H, Rubin BK. MUC5AC and MUC5B mucins increase in cystic fibrosis airway secretions during pulmonary exacerbation. Am J Respir Crit Care Med. 2007;175(8):816-821.
    • (2007) Am J Respir Crit Care Med , vol.175 , Issue.8 , pp. 816-821
    • Henke, M.O.1    John, G.2    Germann, M.3    Lindemann, H.4    Rubin, B.K.5
  • 19
    • 0017105130 scopus 로고
    • Human respiratory tract secretion. Mucous glycoproteins of nonpurulent tracheobronchial secretions, and sputum of patients with bronchitis and cystic fibrosis
    • Boat TF, Cheng PW, Iyer RN, Carlson DM, Polony I. Human respiratory tract secretion. Mucous glycoproteins of nonpurulent tracheobronchial secretions, and sputum of patients with bronchitis and cystic fibrosis. Arch Biochem Biophys. 1976;177(1):95-104.
    • (1976) Arch Biochem Biophys , vol.177 , Issue.1 , pp. 95-104
    • Boat, T.F.1    Cheng, P.W.2    Iyer, R.N.3    Carlson, D.M.4    Polony, I.5
  • 20
    • 0024340091 scopus 로고
    • Evidence for the in vivo degradation of human respiratory mucins during Pseudomonas aeruginosa infection
    • Houdret N, et al. Evidence for the in vivo degradation of human respiratory mucins during Pseudomonas aeruginosa infection. Biochimica Biophys Acta. 1989;992(1):96-105.
    • (1989) Biochimica Biophys Acta , vol.992 , Issue.1 , pp. 96-105
    • Houdret, N.1
  • 21
    • 34548282581 scopus 로고    scopus 로고
    • Sputum biomarkers of inflammation in cystic fibrosis lung disease
    • Sagel SD, Chmiel JF, Konstan MW. Sputum biomarkers of inflammation in cystic fibrosis lung disease. Proc Am Thorac Soc. 2007;4(4):406-417.
    • (2007) Proc Am Thorac Soc , vol.4 , Issue.4 , pp. 406-417
    • Sagel, S.D.1    Chmiel, J.F.2    Konstan, M.W.3
  • 22
    • 84868296268 scopus 로고    scopus 로고
    • Sputum biomarkers of inflammation and lung function decline in children with cystic fibrosis
    • Sagel SD, Wagner BD, Anthony MM, Emmett P, Zemanick ET. Sputum biomarkers of inflammation and lung function decline in children with cystic fibrosis. Am J Respir Crit Care Med. 2012; 186(9):857-865.
    • (2012) Am J Respir Crit Care Med , vol.186 , Issue.9 , pp. 857-865
    • Sagel, S.D.1    Wagner, B.D.2    Anthony, M.M.3    Emmett, P.4    Zemanick, E.T.5
  • 23
    • 0033023581 scopus 로고    scopus 로고
    • Salivary mucin MG1 is comprised almost entirely of different glycosylated forms of the MUC5B gene product
    • Thornton DJ, et al. Salivary mucin MG1 is comprised almost entirely of different glycosylated forms of the MUC5B gene product. Glycobiology. 1999;9(3):293-302.
    • (1999) Glycobiology , vol.9 , Issue.3 , pp. 293-302
    • Thornton, D.J.1
  • 24
    • 44449089926 scopus 로고    scopus 로고
    • Analyzing the functions of large glycoconjugates through the dissipative properties of their absorbed layers using the gelforming mucin MUC5B as an example
    • Kesimer M, Sheehan JK. Analyzing the functions of large glycoconjugates through the dissipative properties of their absorbed layers using the gelforming mucin MUC5B as an example. Glycobiology. 2008;18(6):463-472.
    • (2008) Glycobiology , vol.18 , Issue.6 , pp. 463-472
    • Kesimer, M.1    Sheehan, J.K.2
  • 25
    • 0024340091 scopus 로고
    • Evidence for the in vivo degradation of human respiratory mucins during Pseudomonas aeruginosa infection
    • Houdret N, et al. Evidence for the in vivo degradation of human respiratory mucins during Pseudomonas aeruginosa infection. Biochim Biophys Acta. 1989;992(1):96-105.
    • (1989) Biochim Biophys Acta , vol.992 , Issue.1 , pp. 96-105
    • Houdret, N.1
  • 26
    • 33845309114 scopus 로고    scopus 로고
    • A physical linkage between cystic fibrosis airway surface dehydration and Pseudomonas aeruginosa biofilms
    • Matsui H, et al. A physical linkage between cystic fibrosis airway surface dehydration and Pseudomonas aeruginosa biofilms. Proc Natl Acad Sci U S A. 2006;103(48):18131-18136.
    • (2006) Proc Natl Acad Sci U S A , vol.103 , Issue.48 , pp. 18131-18136
    • Matsui, H.1
  • 27
    • 0030930248 scopus 로고    scopus 로고
    • Roles of Pseudomonas aeruginosa las and rhl quorum-sensing systems in control of elastase and rhamnolipid biosynthesis genes
    • Pearson JP, Pesci EC, Iglewski BH. Roles of Pseudomonas aeruginosa las and rhl quorum-sensing systems in control of elastase and rhamnolipid biosynthesis genes. J Bacteriol. 1997;179(18):5756-5767.
    • (1997) J Bacteriol , vol.179 , Issue.18 , pp. 5756-5767
    • Pearson, J.P.1    Pesci, E.C.2    Iglewski, B.H.3
  • 28
    • 0035676981 scopus 로고    scopus 로고
    • Identification of a nonmucin glycoprotein (gp-340) from a purified respiratory mucin preparation: Evidence for an association involving the MUC5B mucin
    • Thornton DJ, et al. Identification of a nonmucin glycoprotein (gp-340) from a purified respiratory mucin preparation: evidence for an association involving the MUC5B mucin. Glycobiology. 2001;11(11):969-977.
    • (2001) Glycobiology , vol.11 , Issue.11 , pp. 969-977
    • Thornton, D.J.1
  • 29
    • 33744748625 scopus 로고    scopus 로고
    • Regulation of mucin genes in chronic inflammatory airway diseases
    • Voynow JA, Gendler SJ, Rose MC. Regulation of mucin genes in chronic inflammatory airway diseases. Am J Respir Cell Mol Biol. 2006;34(6):661-665.
    • (2006) Am J Respir Cell Mol Biol , vol.34 , Issue.6 , pp. 661-665
    • Voynow, J.A.1    Gendler, S.J.2    Rose, M.C.3
  • 30
    • 24044466139 scopus 로고    scopus 로고
    • 2+i- dependent hyperinflammatory response in human cystic fibrosis airway epithelia
    • Ribeiro CM, et al. Chronic airway infection/inflammation induces a Ca2+i-dependent hyperinflammatory response in human cystic fibrosis airway epithelia. J Biol Chem. 2005;280(18):17798-17806.
    • (2005) J Biol Chem , vol.280 , Issue.18 , pp. 17798-17806
    • Ribeiro, C.M.1
  • 31
    • 84864849180 scopus 로고    scopus 로고
    • Mucus clearance, MyD88-dependent and MyD88-independent immunity modulate lung susceptibility to spontaneous bacterial infection and inflammation
    • Livraghi-Butrico A, et al. Mucus clearance, MyD88-dependent and MyD88-independent immunity modulate lung susceptibility to spontaneous bacterial infection and inflammation. Mucosal Immunol. 2012;5(4):397-408.
    • (2012) Mucosal Immunol , vol.5 , Issue.4 , pp. 397-408
    • Livraghi-Butrico, A.1
  • 32
    • 2942720573 scopus 로고    scopus 로고
    • The nature of small-airway obstruction in chronic obstructive pulmonary disease
    • Hogg JC, et al. The nature of small-airway obstruction in chronic obstructive pulmonary disease. N Engl J Med. 2004;350(26):2645-2653.
    • (2004) N Engl J Med , vol.350 , Issue.26 , pp. 2645-2653
    • Hogg, J.C.1
  • 33
    • 84864591396 scopus 로고    scopus 로고
    • Mapping the protein domain structures of the respiratory mucins: A mucin proteome coverage study
    • Cao R, Wang TT, DeMaria G, Sheehan JK, Kesimer M. Mapping the protein domain structures of the respiratory mucins: a mucin proteome coverage study. J Proteome Res. 2012;11(8):4013-4023.
    • (2012) J Proteome Res , vol.11 , Issue.8 , pp. 4013-4023
    • Cao, R.1    Wang, T.T.2    Demaria, G.3    Sheehan, J.K.4    Kesimer, M.5
  • 34
    • 84878000440 scopus 로고    scopus 로고
    • Risk factors for bronchiectasis in children with cystic fibrosis
    • Sly PD, et al. Risk factors for bronchiectasis in children with cystic fibrosis. N Engl J Med. 2013; 368(21):1963-1970.
    • (2013) N Engl J Med , vol.368 , Issue.21 , pp. 1963-1970
    • Sly, P.D.1
  • 35
    • 58149529356 scopus 로고    scopus 로고
    • Tracheobronchial air-liquid interface cell culture: A model for innate mucosal defense of the upper airways?
    • Kesimer M, et al. Tracheobronchial air-liquid interface cell culture: a model for innate mucosal defense of the upper airways? Am J Physiol Lung Cell Mol Physiol. 2009;296(1):L92-L100.
    • (2009) Am J Physiol Lung Cell Mol Physiol , vol.296 , Issue.1
    • Kesimer, M.1
  • 36
    • 84874102944 scopus 로고    scopus 로고
    • Molecular organization of the mucins and glycocalyx underlying mucus transport over mucosal surfaces of the airways
    • Kesimer M, Ehre C, Burns KA, Davis CW, Sheehan JK, Pickles RJ. Molecular organization of the mucins and glycocalyx underlying mucus transport over mucosal surfaces of the airways. Mucosal Immunol. 2013;6(2):379-392.
    • (2013) Mucosal Immunol , vol.6 , Issue.2 , pp. 379-392
    • Kesimer, M.1    Ehre, C.2    Burns, K.A.3    Davis, C.W.4    Sheehan, J.K.5    Pickles, R.J.6
  • 37
    • 34047166052 scopus 로고    scopus 로고
    • Airway surface dehydration in cystic fibrosis: Pathogenesis and therapy
    • Boucher RC. Airway surface dehydration in cystic fibrosis: pathogenesis and therapy. Annu Rev Med. 2007;58:157-170.
    • (2007) Annu Rev Med , vol.58 , pp. 157-170
    • Boucher, R.C.1
  • 38
    • 33750603848 scopus 로고    scopus 로고
    • Characterizing mucous cell remodeling in cystic fibrosis: Relationship to neutrophils
    • Hays SR, Fahy JV. Characterizing mucous cell remodeling in cystic fibrosis: relationship to neutrophils. Am J Respir Crit Care Med. 2006; 174(9):1018-1024.
    • (2006) Am J Respir Crit Care Med , vol.174 , Issue.9 , pp. 1018-1024
    • Hays, S.R.1    Fahy, J.V.2
  • 39
    • 0014862093 scopus 로고
    • Sputum viscosity in cystic fibrosis of the pancreas and other pulmonary diseases
    • Feather EA, Russell G. Sputum viscosity in cystic fibrosis of the pancreas and other pulmonary diseases. Br J Dis Chest. 1970;64(4):192-200.
    • (1970) Br J Dis Chest , vol.64 , Issue.4 , pp. 192-200
    • Feather, E.A.1    Russell, G.2
  • 40
    • 0032770489 scopus 로고    scopus 로고
    • Quantitation of inflammatory responses to bacteria in young cystic fibrosis and control patients
    • Muhlebach MS, Stewart PW, Leigh MW, Noah TL. Quantitation of inflammatory responses to bacteria in young cystic fibrosis and control patients. Am J Respir Crit Care Med. 1999;160(1):186-191.
    • (1999) Am J Respir Crit Care Med , vol.160 , Issue.1 , pp. 186-191
    • Muhlebach, M.S.1    Stewart, P.W.2    Leigh, M.W.3    Noah, T.L.4
  • 42
    • 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
    • Holmen, J.M.1
  • 44
    • 0345257066 scopus 로고    scopus 로고
    • New monoclonal antibodies to non-glycosylated domains of the secreted mucins MUC5B and MUC7
    • Rousseau K, Wickstrom C, Whitehouse DB, Carlstedt I, Swallow DM. New monoclonal antibodies to non-glycosylated domains of the secreted mucins MUC5B and MUC7. Hybrid Hybridomics. 2003;22(5):293-299.
    • (2003) Hybrid Hybridomics , vol.22 , Issue.5 , pp. 293-299
    • Rousseau, K.1    Wickstrom, C.2    Whitehouse, D.B.3    Carlstedt, I.4    Swallow, D.M.5
  • 45
    • 84856324259 scopus 로고    scopus 로고
    • Mass spectrometric analysis of mucin core proteins
    • Kesimer M, Sheehan JK. Mass spectrometric analysis of mucin core proteins. Methods Mol Biol. 2012;842:67-79.
    • (2012) Methods Mol Biol , vol.842 , pp. 67-79
    • Kesimer, M.1    Sheehan, J.K.2
  • 46
    • 0028310723 scopus 로고
    • Identification of glycoproteins on nitrocellulose membranes and gels
    • Thornton DJ, Carlstedt I, Sheehan JK. Identification of glycoproteins on nitrocellulose membranes and gels. Methods Mol Biol. 1994;32:119-128.
    • (1994) Methods Mol Biol , vol.32 , pp. 119-128
    • Thornton, D.J.1    Carlstedt, I.2    Sheehan, J.K.3
  • 47
    • 0030030161 scopus 로고    scopus 로고
    • Rapid size distribution and purity analysis of gastric mucus glycoproteins by size exclusion chromatography/multi angle laser light scattering
    • Jumel K, Fiebrig I, Harding SE. Rapid size distribution and purity analysis of gastric mucus glycoproteins by size exclusion chromatography/multi angle laser light scattering. Int J Biol Macromol. 1996;18(1-2):133-139.
    • (1996) Int J Biol Macromol , vol.18 , Issue.1-2 , pp. 133-139
    • Jumel, K.1    Fiebrig, I.2    Harding, S.E.3
  • 48
    • 38149098668 scopus 로고    scopus 로고
    • Mapping of the 45M1 epitope to the C-terminal cysteine-rich part of the human MUC5AC mucin
    • Lidell ME, Bara J, Hansson GC. Mapping of the 45M1 epitope to the C-terminal cysteine-rich part of the human MUC5AC mucin. FEBS J. 2008;275(3):481-489.
    • (2008) FEBS J , vol.275 , Issue.3 , pp. 481-489
    • Lidell, M.E.1    Bara, J.2    Hansson, G.C.3
  • 49
    • 45549102949 scopus 로고    scopus 로고
    • Munc13-2-/-baseline secretion defect reveals source of oligomeric mucins in mouse airways
    • Zhu Y, et al. Munc13-2-/-baseline secretion defect reveals source of oligomeric mucins in mouse airways. J Physiol. 2008;586(7):1977-1992.
    • (2008) J Physiol , vol.586 , Issue.7 , pp. 1977-1992
    • Zhu, Y.1


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.