메뉴 건너뛰기




Volumn 35, Issue 1, 2007, Pages 116-129

Cystic Fibrosis and Other Respiratory Diseases of Impaired Mucus Clearance

Author keywords

Chronic obstructive pulmonary disease; cilia; cystic fibrosis; mucin; mucociliary clearance; primary ciliary dyskinesia

Indexed keywords

CYTOKINE; DNA POLYMERASE; DNA POLYMERASE LAMBDA; MUCIN; PROTEIN; PROTEIN HFH4; PROTEIN MDHC7; PROTEIN MDNAH5; PROTEIN SCNN1B; TRANSMEMBRANE CONDUCTANCE REGULATOR; UNCLASSIFIED DRUG;

EID: 33847289649     PISSN: 01926233     EISSN: None     Source Type: Journal    
DOI: 10.1080/01926230601060025     Document Type: Article
Times cited : (154)

References (90)
  • 1
    • 0017162819 scopus 로고
    • A human syndrome caused by immotile cilia
    • Afzelius BA A human syndrome caused by immotile cilia Science 1976 193 317 9
    • (1976) Science , vol.193 , pp. 317-319
    • Afzelius, B.A.1
  • 2
    • 7944223873 scopus 로고    scopus 로고
    • Cilia-related diseases
    • Afzelius BA Cilia-related diseases J Pathol 2004 204 470 7
    • (2004) J Pathol , vol.204 , pp. 470-477
    • Afzelius, B.A.1
  • 5
    • 0024459145 scopus 로고
    • Complement receptor expression on neutrophils at an inflammatory site, the Pseudomonas-infected lung in cystic fibrosis
    • Berger M Sorensen RU Tosi MF Dearborn DG Doring G Complement receptor expression on neutrophils at an inflammatory site, the Pseudomonas-infected lung in cystic fibrosis J Clin Invest 1989 84 1302 13
    • (1989) J Clin Invest , vol.84 , pp. 1302-1313
    • Berger, M.1    Sorensen, R.U.2    Tosi, M.F.3    Dearborn, D.G.4    Doring, G.5
  • 10
    • 33745763288 scopus 로고    scopus 로고
    • Movement: the emerging genetics of primary ciliary dyskinesia
    • Bush A Ferkol T Movement: the emerging genetics of primary ciliary dyskinesia Am J Respir Crit Care Med 2006 174 109 10
    • (2006) Am J Respir Crit Care Med , vol.174 , pp. 109-110
    • Bush, A.1    Ferkol, T.2
  • 13
    • 32244433770 scopus 로고    scopus 로고
    • The physiology of cough
    • Chang AB The physiology of cough Paediatr Respir Rev 2006 7 2 8
    • (2006) Paediatr Respir Rev , vol.7 , pp. 2-8
    • Chang, A.B.1
  • 14
    • 0032530312 scopus 로고    scopus 로고
    • Mutation of the mouse hepatocyte nuclear factor/forkhead homologue 4 gene results in an absence of cilia and random left-right asymmetry
    • Chen J Knowles HJ Hebert JL Hackett BP Mutation of the mouse hepatocyte nuclear factor/forkhead homologue 4 gene results in an absence of cilia and random left-right asymmetry J Clin Invest 1998 102 1077 82
    • (1998) J Clin Invest , vol.102 , pp. 1077-1082
    • Chen, J.1    Knowles, H.J.2    Hebert, J.L.3    Hackett, B.P.4
  • 16
    • 27944469172 scopus 로고    scopus 로고
    • An incredible decade for the primary cilium: a look at a once-forgotten organelle
    • Davenport JR Yoder BK An incredible decade for the primary cilium: a look at a once-forgotten organelle Am J Physiol Renal Physiol 2005 289 F1159 69
    • (2005) Am J Physiol Renal Physiol , vol.289 , pp. F1159-F1169
    • Davenport, J.R.1    Yoder, B.K.2
  • 17
  • 19
    • 1642277855 scopus 로고    scopus 로고
    • Characteristic multiorgan pathology of cystic fibrosis in a long-living cystic fibrosis trans-membrane regulator knockout murine model
    • Durie PR Kent G Phillips MJ Ackerley CA Characteristic multiorgan pathology of cystic fibrosis in a long-living cystic fibrosis trans-membrane regulator knockout murine model Am J Pathol 2004 164 1481 93
    • (2004) Am J Pathol , vol.164 , pp. 1481-1493
    • Durie, P.R.1    Kent, G.2    Phillips, M.J.3    Ackerley, C.A.4
  • 23
    • 33646148702 scopus 로고    scopus 로고
    • Novel tools to unravel molecular mechanisms in cilia-related disorders
    • Fliegauf M Omran H Novel tools to unravel molecular mechanisms in cilia-related disorders Trends Genet 2006 22 241 5
    • (2006) Trends Genet , vol.22 , pp. 241-245
    • Fliegauf, M.1    Omran, H.2
  • 24
    • 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 109 693 7
    • (2002) J Clin Invest , vol.109 , pp. 693-697
    • Ganz, T.1
  • 25
    • 4644225352 scopus 로고    scopus 로고
    • Primary ciliary dyskinesia: genes, candidate genes and chromosomal regions
    • Geremek M Witt M Primary ciliary dyskinesia: genes, candidate genes and chromosomal regions J Appl Genet 2004 45 347 61
    • (2004) J Appl Genet , vol.45 , pp. 347-361
    • Geremek, M.1    Witt, M.2
  • 26
    • 0032962320 scopus 로고    scopus 로고
    • Pathophysiology of gene-targeted mouse models for cystic fibrosis
    • Grubb BR Boucher RC Pathophysiology of gene-targeted mouse models for cystic fibrosis Physiol Rev 1999 79 S193 214
    • (1999) Physiol Rev , vol.79 , pp. S193-S214
    • Grubb, B.R.1    Boucher, R.C.2
  • 27
    • 0028111842 scopus 로고
    • Anomalies in ion transport in CF mouse tracheal epithelium
    • Grubb BR Paradiso AM Boucher RC Anomalies in ion transport in CF mouse tracheal epithelium Am J Physiol 1994 267 C293 300
    • (1994) Am J Physiol , vol.267 , pp. C293-C300
    • Grubb, B.R.1    Paradiso, A.M.2    Boucher, R.C.3
  • 29
    • 33646204913 scopus 로고    scopus 로고
    • Nodal flow and the generation of left-right asymmetry
    • Hirokawa N Tanaka Y Okada Y Takeda S Nodal flow and the generation of left-right asymmetry Cell 2006 125 33 45
    • (2006) Cell , vol.125 , pp. 33-45
    • Hirokawa, N.1    Tanaka, Y.2    Okada, Y.3    Takeda, S.4
  • 32
    • 0037087624 scopus 로고    scopus 로고
    • Loss of function of axonemal dynein Mdnah5 causes primary ciliary dyskinesia and hydrocephalus
    • Ibanez-Tallon I Gorokhova S Heintz N Loss of function of axonemal dynein Mdnah5 causes primary ciliary dyskinesia and hydrocephalus Hum Mol Genet 2002 11 715 21
    • (2002) Hum Mol Genet , vol.11 , pp. 715-721
    • Ibanez-Tallon, I.1    Gorokhova, S.2    Heintz, N.3
  • 33
    • 1642564276 scopus 로고    scopus 로고
    • To beat or not to beat: roles of cilia in development and disease
    • Ibanez-Tallon I Heintz N Omran H To beat or not to beat: roles of cilia in development and disease Hum Mol Genet 2003 12 Spec1 R27 35
    • (2003) Hum Mol Genet , vol.12 , pp. R27-R35
    • Ibanez-Tallon, I.1    Heintz, N.2    Omran, H.3
  • 34
    • 26444432400 scopus 로고    scopus 로고
    • Cystic fibrosis and airway submucosal glands
    • Inglis SK Wilson SM Cystic fibrosis and airway submucosal glands Pediatr Pulmonol 2005 40 279 84
    • (2005) Pediatr Pulmonol , vol.40 , pp. 279-284
    • Inglis, S.K.1    Wilson, S.M.2
  • 35
    • 0034157665 scopus 로고    scopus 로고
    • Ciliary function analysis for the diagnosis of primary ciliary dyskinesia: advantages of ciliogenesis in culture
    • Jorissen M Willems T Van der Schueren B Ciliary function analysis for the diagnosis of primary ciliary dyskinesia: advantages of ciliogenesis in culture Acta Otolaryngol 2000 120 291 5
    • (2000) Acta Otolaryngol , vol.120 , pp. 291-295
    • Jorissen, M.1    Willems, T.2    Van der Schueren, B.3
  • 36
    • 7644242159 scopus 로고
    • Situs viscerum inversus und Polyposis nasi in einem Falle familiaerer Bronchiektasien
    • Kartagener MHA Situs viscerum inversus und Polyposis nasi in einem Falle familiaerer Bronchiektasien Beitr Klin Tuberk 1936 331 3
    • (1936) Beitr Klin Tuberk , pp. 331-333
    • Kartagener, M.H.A.1
  • 38
    • 0036194724 scopus 로고    scopus 로고
    • Mucus clearance as a primary innate defense mechanism for mammalian airways
    • Knowles MR Boucher RC Mucus clearance as a primary innate defense mechanism for mammalian airways J Clin Invest 2002 109 571 7
    • (2002) J Clin Invest , vol.109 , pp. 571-577
    • Knowles, M.R.1    Boucher, R.C.2
  • 39
    • 17944397758 scopus 로고    scopus 로고
    • Hydrocephalus, situs inversus, chronic sinusitis, and male infertility in dna polymerase lambda-deficient mice: possible implication for the pathogenesis of immotile cilia syndrome
    • Kobayashi Y Watanabe M Okada Y Sawa H Takai H Nakanishi M Kawase Y Suzuki H Nagashima K Ikeda K Motoyama N Hydrocephalus, situs inversus, chronic sinusitis, and male infertility in dna polymerase lambda-deficient mice: possible implication for the pathogenesis of immotile cilia syndrome Mol Cell Biol 2002 22 2769 76
    • (2002) Mol Cell Biol , vol.22 , pp. 2769-2776
    • Kobayashi, Y.1    Watanabe, M.2    Okada, Y.3    Sawa, H.4    Takai, H.5    Nakanishi, M.6    Kawase, Y.7    Suzuki, H.8    Nagashima, K.9    Ikeda, K.10    Motoyama, N.11
  • 43
    • 33746583563 scopus 로고    scopus 로고
    • Innate immune response in CF airway epithelia: hyper-inflammatory?
    • Machen TE Innate immune response in CF airway epithelia: hyper-inflammatory? Am J Physiol Cell Physiol 2006 291 C218 30
    • (2006) Am J Physiol Cell Physiol , vol.291 , pp. C218-C230
    • Machen, T.E.1
  • 45
    • 2442718786 scopus 로고    scopus 로고
    • + absorption produces cystic fibrosis-like lung disease in mice
    • 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 93
    • (2004) Nat Med , vol.10 , pp. 487-493
    • Mall, M.1    Grubb, B.R.2    Harkema, J.R.3    O’Neal, W.K.4    Boucher, R.C.5
  • 47
    • 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 BR Tarran R Randell SH Gatzy JT Davis CW Boucher RC Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease Cell 1998 95 1005 15
    • (1998) Cell , vol.95 , pp. 1005-1015
    • Matsui, H.1    Grubb, B.R.2    Tarran, R.3    Randell, S.H.4    Gatzy, J.T.5    Davis, C.W.6    Boucher, R.C.7
  • 48
    • 0031013538 scopus 로고    scopus 로고
    • Loss of binding and entry of liposome-DNA complexes decreases transfection efficiency in differentiated airway epithelial cells
    • Matsui H Johnson LG Randell SH Boucher RC Loss of binding and entry of liposome-DNA complexes decreases transfection efficiency in differentiated airway epithelial cells J Biol Chem 1997 272 1117 26
    • (1997) J Biol Chem , vol.272 , pp. 1117-1126
    • Matsui, H.1    Johnson, L.G.2    Randell, S.H.3    Boucher, R.C.4
  • 49
    • 23244461620 scopus 로고    scopus 로고
    • Reduced three-dimensional motility in dehydrated airway mucus prevents neutrophil capture and killing bacteria on airway epithelial surfaces
    • Matsui H Verghese MW Kesimer M Schwab UE Randell SH Sheehan JK Grubb BR Boucher RC Reduced three-dimensional motility in dehydrated airway mucus prevents neutrophil capture and killing bacteria on airway epithelial surfaces J Immunol 2005 175 1090 9
    • (2005) J Immunol , vol.175 , pp. 1090-1099
    • Matsui, H.1    Verghese, M.W.2    Kesimer, M.3    Schwab, U.E.4    Randell, S.H.5    Sheehan, J.K.6    Grubb, B.R.7    Boucher, R.C.8
  • 50
    • 33144458257 scopus 로고    scopus 로고
    • Global physiology and pathophysiology of cough: ACCP evidence-based clinical practice guidelines
    • McCool FD Global physiology and pathophysiology of cough: ACCP evidence-based clinical practice guidelines Chest 2006 129 48S 53S
    • (2006) Chest , vol.129 , pp. 48S-53S
    • McCool, F.D.1
  • 51
    • 0034107313 scopus 로고    scopus 로고
    • Primary ciliary dyskinesia (PCD)
    • Meeks M Bush A Primary ciliary dyskinesia (PCD) Pediatr Pulmonol 2000 29 307 16
    • (2000) Pediatr Pulmonol , vol.29 , pp. 307-316
    • Meeks, M.1    Bush, A.2
  • 57
    • 0019364354 scopus 로고
    • The cells of the tracheo-bronchial epithelium of the mouse: a quantitative light and electron microscope study
    • Pack RJ Al-Ugaily LH Morris G The cells of the tracheo-bronchial epithelium of the mouse: a quantitative light and electron microscope study J Anat 1981 132 71 84
    • (1981) J Anat , vol.132 , pp. 71-84
    • Pack, R.J.1    Al-Ugaily, L.H.2    Morris, G.3
  • 58
    • 0034255014 scopus 로고    scopus 로고
    • Role of the cystic fibrosis transmembrane conductance regulator in innate immunity to Pseudomonas aeruginosa infections
    • Pier GB Role of the cystic fibrosis transmembrane conductance regulator in innate immunity to Pseudomonas aeruginosa infections Proc Natl Acad Sci USA 2000 97 8822 8
    • (2000) Proc Natl Acad Sci USA , vol.97 , pp. 8822-8828
    • Pier, G.B.1
  • 59
    • 33745595399 scopus 로고    scopus 로고
    • Effective mucus clearance is essential for respiratory health
    • Randell SH Boucher RC Effective mucus clearance is essential for respiratory health Am J Respir Cell Mol Biol 2006 35 20 8
    • (2006) Am J Respir Cell Mol Biol , vol.35 , pp. 20-28
    • Randell, S.H.1    Boucher, R.C.2
  • 60
    • 0027732178 scopus 로고
    • Immotile cilia syndrome in pigs. A model for human disease
    • Roperto F Galati P Rossacco P Immotile cilia syndrome in pigs. A model for human disease Am J Pathol 1993 143 643 7
    • (1993) Am J Pathol , vol.143 , pp. 643-647
    • Roperto, F.1    Galati, P.2    Rossacco, P.3
  • 61
    • 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 86 245 78
    • (2006) Physiol Rev , vol.86 , pp. 245-278
    • Rose, M.C.1    Voynow, J.A.2
  • 63
    • 0027428606 scopus 로고
    • Pseudomonas aeruginosa pili bind to asialoGM1 which is increased on the surface of cystic fibrosis epithelial cells
    • Saiman L Prince A Pseudomonas aeruginosa pili bind to asialoGM1 which is increased on the surface of cystic fibrosis epithelial cells J Clin Invest 1993 92 1875 80
    • (1993) J Clin Invest , vol.92 , pp. 1875-1880
    • Saiman, L.1    Prince, A.2
  • 64
    • 33646164168 scopus 로고    scopus 로고
    • Intraflagellar transport and cilium-based signaling
    • Scholey JM Anderson KV Intraflagellar transport and cilium-based signaling Cell 2006 125 439 42
    • (2006) Cell , vol.125 , pp. 439-442
    • Scholey, J.M.1    Anderson, K.V.2
  • 66
    • 85047681474 scopus 로고    scopus 로고
    • Assembly and motility of eukaryotic cilia and flagella. Lessons from Chlamydomonas reinhardtii
    • Silflow CD Lefebvre PA Assembly and motility of eukaryotic cilia and flagella. Lessons from Chlamydomonas reinhardtii Plant Physiol 2001 127 1500 7
    • (2001) Plant Physiol , vol.127 , pp. 1500-1507
    • Silflow, C.D.1    Lefebvre, P.A.2
  • 68
    • 33746891890 scopus 로고    scopus 로고
    • The primary cilium as the cell's antenna: signaling at a sensory organelle
    • Singla V Reiter JF The primary cilium as the cell's antenna: signaling at a sensory organelle Science 2006 313 629 33
    • (2006) Science , vol.313 , pp. 629-633
    • Singla, V.1    Reiter, J.F.2
  • 71
    • 33645464522 scopus 로고    scopus 로고
    • Ciliary function and the role of cilia in clearance
    • Stannard W O’Callaghan C Ciliary function and the role of cilia in clearance J Aerosol Med 2006 19 110 5
    • (2006) J Aerosol Med , vol.19 , pp. 110-115
    • Stannard, W.1    O’Callaghan, C.2
  • 72
    • 0033377234 scopus 로고    scopus 로고
    • Targeted deletion of the ATP binding domain of left-right dynein confirms its role in specifying development of left-right asymmetries
    • Supp DM Brueckner M Kuehn MR Witte DP Lowe LA McGrath J Corrales J Potter SS Targeted deletion of the ATP binding domain of left-right dynein confirms its role in specifying development of left-right asymmetries Development 1999 126 5495 504
    • (1999) Development , vol.126 , pp. 5495-5504
    • Supp, D.M.1    Brueckner, M.2    Kuehn, M.R.3    Witte, D.P.4    Lowe, L.A.5    McGrath, J.6    Corrales, J.7    Potter, S.S.8
  • 74
    • 11144240244 scopus 로고    scopus 로고
    • Regulation of airway surface liquid volume and mucus transport by active ion transport
    • Tarran R Regulation of airway surface liquid volume and mucus transport by active ion transport Proc Am Thorac Soc 2004 1 42 6
    • (2004) Proc Am Thorac Soc , vol.1 , pp. 42-46
    • Tarran, R.1
  • 75
    • 0036012529 scopus 로고    scopus 로고
    • Thin-film measurements of airway surface liquid volume/composition and mucus transport rates in vitro
    • Tarran R Boucher RC Thin-film measurements of airway surface liquid volume/composition and mucus transport rates in vitro Methods Mol Med 2002 70 479 92
    • (2002) Methods Mol Med , vol.70 , pp. 479-492
    • Tarran, R.1    Boucher, R.C.2
  • 76
    • 33645964965 scopus 로고    scopus 로고
    • Regulation of normal and cystic fibrosis airway surface liquid volume by phasic shear stress
    • Tarran R Button B Boucher RC Regulation of normal and cystic fibrosis airway surface liquid volume by phasic shear stress Annu Rev Physiol 2006a 68 543 61
    • (2006) Annu Rev Physiol , vol.68 , pp. 543-561
    • Tarran, R.1    Button, B.2    Boucher, R.C.3
  • 79
    • 33646138489 scopus 로고    scopus 로고
    • Soluble mediators, not cilia, determine airway surface liquid volume in normal and cystic fibrosis superficial airway epithelia
    • Tarran R Trout L Donaldson SH Boucher RC Soluble mediators, not cilia, determine airway surface liquid volume in normal and cystic fibrosis superficial airway epithelia J Gen Physiol 2006b 127 591 604
    • (2006) J Gen Physiol , vol.127 , pp. 591-604
    • Tarran, R.1    Trout, L.2    Donaldson, S.H.3    Boucher, R.C.4
  • 80
    • 7044270542 scopus 로고    scopus 로고
    • From mucins to mucus: toward a more coherent understanding of this essential barrier. 10.1513/pats.2306016
    • Thornton DJ Sheehan JK From mucins to mucus: toward a more coherent understanding of this essential barrier. 10.1513/pats.2306016 Proc Am Thorac Soc 2004 1 54 61
    • (2004) Proc Am Thorac Soc , vol.1 , pp. 54-61
    • Thornton, D.J.1    Sheehan, J.K.2
  • 81
    • 26944440889 scopus 로고    scopus 로고
    • Primary ciliary dyskinesia: clinical presentation, diagnosis and genetics
    • Van's Gravesande KS Omran H Primary ciliary dyskinesia: clinical presentation, diagnosis and genetics Ann Med 2005 37 439 49
    • (2005) Ann Med , vol.37 , pp. 439-449
    • Van's Gravesande, K.S.1    Omran, H.2
  • 82
    • 19944396801 scopus 로고    scopus 로고
    • Further studies on knockout mice lacking a functional dynein heavy chain (MDHC7). 1. Evidence for a structural deficit in the axoneme
    • Vernon GG Neesen J Woolley DM Further studies on knockout mice lacking a functional dynein heavy chain (MDHC7). 1. Evidence for a structural deficit in the axoneme Cell Motil Cytoskeleton 2005 61 65 73
    • (2005) Cell Motil Cytoskeleton , vol.61 , pp. 65-73
    • Vernon, G.G.1    Neesen, J.2    Woolley, D.M.3
  • 83
    • 0037067169 scopus 로고    scopus 로고
    • Respiratory infections with Pseudomonas aeruginosa in children with cystic fibrosis: early detection by serology and assessment of risk factors
    • West SE Zeng L Lee BL Kosorok MR Laxova A Rock MJ Splaingard MJ Farrell PM Respiratory infections with Pseudomonas aeruginosa in children with cystic fibrosis: early detection by serology and assessment of risk factors JAMA 2002 287 2958 67
    • (2002) JAMA , vol.287 , pp. 2958-2967
    • West, S.E.1    Zeng, L.2    Lee, B.L.3    Kosorok, M.R.4    Laxova, A.5    Rock, M.J.6    Splaingard, M.J.7    Farrell, P.M.8
  • 85
    • 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 47 53
    • (2004) Proc Am Thorac Soc , vol.1 , pp. 47-53
    • Wine, J.J.1    Joo, N.S.2
  • 86
    • 19944412178 scopus 로고    scopus 로고
    • Further studies on knockout mice lacking a functional dynein heavy chain (MDHC7). 2. A developmental explanation for the asthenozoospermia
    • Woolley DM Neesen J Vernon GG Further studies on knockout mice lacking a functional dynein heavy chain (MDHC7). 2. A developmental explanation for the asthenozoospermia Cell Motil Cytoskeleton 2005 61 74 82
    • (2005) Cell Motil Cytoskeleton , vol.61 , pp. 74-82
    • Woolley, D.M.1    Neesen, J.2    Vernon, G.G.3
  • 88
    • 25144453883 scopus 로고    scopus 로고
    • Innate immunity and the lung: defense at the interface between host and environment
    • Zaas AK Schwartz DA Innate immunity and the lung: defense at the interface between host and environment Trends Cardiovasc Med 2005 15 195 202
    • (2005) Trends Cardiovasc Med , vol.15 , pp. 195-202
    • Zaas, A.K.1    Schwartz, D.A.2


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.