-
1
-
-
0017162819
-
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
-
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
-
4
-
-
0036678117
-
Mutations in the DNAH11 (axonemal heavy chain dynein type 11) gene cause one form of situs inversus totalis and most likely primary ciliary dyskinesia
-
Bartoloni L Blouin JL Pan Y Gehrig C Maiti AK Scamuffa N Rossier C Jorissen M Armengot M Meeks M Mitchison HM Chung EM Delozier-Blanchet CD Craigen WJ Antonarakis SE Mutations in the DNAH11 (axonemal heavy chain dynein type 11) gene cause one form of situs inversus totalis and most likely primary ciliary dyskinesia Proc Natl Acad Sci USA 2002 99 10282 6
-
(2002)
Proc Natl Acad Sci USA
, vol.99
, pp. 10282-10286
-
-
Bartoloni, L.1
Blouin, J.L.2
Pan, Y.3
Gehrig, C.4
Maiti, A.K.5
Scamuffa, N.6
Rossier, C.7
Jorissen, M.8
Armengot, M.9
Meeks, M.10
Mitchison, H.M.11
Chung, E.M.12
Delozier-Blanchet, C.D.13
Craigen, W.J.14
Antonarakis, S.E.15
-
5
-
-
0024459145
-
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
-
6
-
-
0037090311
-
Cutting edge: DNA polymerases micro and lambda are dispensable for Ig gene hypermutation
-
Bertocci B De Smet A Flatter E Dahan A Bories J-C Landreau C Weill J-C Reynaud C-A Cutting edge: DNA polymerases micro and lambda are dispensable for Ig gene hypermutation J Immunol 2002 168 3702 6
-
(2002)
J Immunol
, vol.168
, pp. 3702-3706
-
-
Bertocci, B.1
De Smet, A.2
Flatter, E.3
Dahan, A.4
Bories, J.-C.5
Landreau, C.6
Weill, J.-C.7
Reynaud, C.-A.8
-
9
-
-
0035253464
-
Longitudinal assessment of Pseudomonas aeruginosa in young children with cystic fibrosis
-
Burns JL Gibson RL McNamara S Yim D Emerson J Rosenfeld M Hiatt P McCoy K Castile R Smith AL Ramsey BW Longitudinal assessment of Pseudomonas aeruginosa in young children with cystic fibrosis J Infect Dis 2001 183 444 52
-
(2001)
J Infect Dis
, vol.183
, pp. 444-452
-
-
Burns, J.L.1
Gibson, R.L.2
McNamara, S.3
Yim, D.4
Emerson, J.5
Rosenfeld, M.6
Hiatt, P.7
McCoy, K.8
Castile, R.9
Smith, A.L.10
Ramsey, B.W.11
-
10
-
-
33745763288
-
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
-
11
-
-
0035121848
-
Ultrastructural nasal pathology in children chronically and sequentially exposed to air pollutants
-
Calderon-Garciduenas L Valencia-Salazar G Rodriguez-Alcaraz A Gambling TM Garcia R Osnaya N Villarreal-Calderon A Devlin RB Carson JL Ultrastructural nasal pathology in children chronically and sequentially exposed to air pollutants Am J Respir Cell Mol Biol 2001 24 132 8
-
(2001)
Am J Respir Cell Mol Biol
, vol.24
, pp. 132-138
-
-
Calderon-Garciduenas, L.1
Valencia-Salazar, G.2
Rodriguez-Alcaraz, A.3
Gambling, T.M.4
Garcia, R.5
Osnaya, N.6
Villarreal-Calderon, A.7
Devlin, R.B.8
Carson, J.L.9
-
12
-
-
33646540731
-
Cystic fibrosis transmembrane conductance regulator function is suppressed in cigarette smokers
-
Cantin AM Hanrahan JW Bilodeau G Ellis L Dupuis A Liao J Zielenski J Durie P Cystic fibrosis transmembrane conductance regulator function is suppressed in cigarette smokers Am J Respir Crit Care Med 2006 173 1139 44
-
(2006)
Am J Respir Crit Care Med
, vol.173
, pp. 1139-1144
-
-
Cantin, A.M.1
Hanrahan, J.W.2
Bilodeau, G.3
Ellis, L.4
Dupuis, A.5
Liao, J.6
Zielenski, J.7
Durie, P.8
-
13
-
-
32244433770
-
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
-
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
-
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
-
-
33645130994
-
Cystic fibrosis since 1938
-
Davis PB Cystic fibrosis since 1938 Am J Respir Crit Care Med 2006 173 475 82
-
(2006)
Am J Respir Crit Care Med
, vol.173
, pp. 475-482
-
-
Davis, P.B.1
-
18
-
-
9044236531
-
Cystic fibrosis mice carrying the missense mutation G551D replicate human genotype-phenotype correlations
-
Delaney SJ Alton EW Smith SN Lunn DP Farley R Lovelock PK Thomson SA Hume DA Lamb D Porteous DJ Dorin JR Wainwright BJ Cystic fibrosis mice carrying the missense mutation G551D replicate human genotype-phenotype correlations Embo J 1996 15 955 63
-
(1996)
Embo J
, vol.15
, pp. 955-963
-
-
Delaney, S.J.1
Alton, E.W.2
Smith, S.N.3
Lunn, D.P.4
Farley, R.5
Lovelock, P.K.6
Thomson, S.A.7
Hume, D.A.8
Lamb, D.9
Porteous, D.J.10
Dorin, J.R.11
Wainwright, B.J.12
-
19
-
-
1642277855
-
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
-
22
-
-
20444364841
-
Mislocalization of DNAH5 and DNAH9 in respiratory cells from patients with primary ciliary dyskinesia
-
Fliegauf M Olbrich H Horvath J Wildhaber JH Zariwala MA Kennedy M Knowles MR Omran H Mislocalization of DNAH5 and DNAH9 in respiratory cells from patients with primary ciliary dyskinesia Am J Respir Crit Care Med 2005 171 1343 9
-
(2005)
Am J Respir Crit Care Med
, vol.171
, pp. 1343-1349
-
-
Fliegauf, M.1
Olbrich, H.2
Horvath, J.3
Wildhaber, J.H.4
Zariwala, M.A.5
Kennedy, M.6
Knowles, M.R.7
Omran, H.8
-
23
-
-
33646148702
-
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
-
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
-
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
-
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
-
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
-
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
-
30
-
-
2942720573
-
The nature of small-airway obstruction in chronic obstructive pulmonary disease
-
Hogg JC Chu F Utokaparch S Woods R Elliott WM Buzatu L Cherniack RM Rogers RM Sciurba FC Coxson HO Pare PD The nature of small-airway obstruction in chronic obstructive pulmonary disease N Engl J Med 2004 350 2645 53
-
(2004)
N Engl J Med
, vol.350
, pp. 2645-2653
-
-
Hogg, J.C.1
Chu, F.2
Utokaparch, S.3
Woods, R.4
Elliott, W.M.5
Buzatu, L.6
Cherniack, R.M.7
Rogers, R.M.8
Sciurba, F.C.9
Coxson, H.O.10
Pare, P.D.11
-
31
-
-
33745748480
-
DNAH5 mutations are a common cause of primary ciliary dyskinesia with outer Dynein arm defects
-
Hornef N Olbrich H Horvath J Zariwala MA Fliegauf M Loges NT Wildhaber J Noone PG Kennedy M Antonarakis SE Blouin JL Bartoloni L Nublein T Ahrens P Griese M Kuhl H Sudbrak R Knowles MR Reinhardt R Omran H DNAH5 mutations are a common cause of primary ciliary dyskinesia with outer Dynein arm defects Am J Respir Crit Care Med 2006
-
(2006)
Am J Respir Crit Care Med
-
-
Hornef, N.1
Olbrich, H.2
Horvath, J.3
Zariwala, M.A.4
Fliegauf, M.5
Loges, N.T.6
Wildhaber, J.7
Noone, P.G.8
Kennedy, M.9
Antonarakis, S.E.10
Blouin, J.L.11
Bartoloni, L.12
Nublein, T.13
Ahrens, P.14
Griese, M.15
Kuhl, H.16
Sudbrak, R.17
Knowles, M.R.18
Reinhardt, R.19
Omran, H.20
more..
-
32
-
-
0037087624
-
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
-
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
-
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
-
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
-
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
-
37
-
-
0031438318
-
Lung disease in mice with cystic fibrosis
-
Kent G Iles R Bear CE Huan L-J Griesenbach U McKerlie C Frndova H Ackerley C Gosselin D Radzioch D O’Brodovich H Tsui L-C Buchwald M Tanswell AK Lung disease in mice with cystic fibrosis J Clin Invest 1997 100 3060 9
-
(1997)
J Clin Invest
, vol.100
, pp. 3060-3069
-
-
Kent, G.1
Iles, R.2
Bear, C.E.3
Huan, L.-J.4
Griesenbach, U.5
McKerlie, C.6
Frndova, H.7
Ackerley, C.8
Gosselin, D.9
Radzioch, D.10
O’Brodovich, H.11
Tsui, L.-C.12
Buchwald, M.13
Tanswell, A.K.14
-
38
-
-
0036194724
-
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
-
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
-
42
-
-
33646198140
-
Cloned ferrets produced by somatic cell nuclear transfer
-
Li Z Sun X Chen J Liu X Wisely SM Zhou Q Renard JP Leno GH Engelhardt JF Cloned ferrets produced by somatic cell nuclear transfer Dev Biol 2006 293 439 48
-
(2006)
Dev Biol
, vol.293
, pp. 439-448
-
-
Li, Z.1
Sun, X.2
Chen, J.3
Liu, X.4
Wisely, S.M.5
Zhou, Q.6
Renard, J.P.7
Leno, G.H.8
Engelhardt, J.F.9
-
43
-
-
33746583563
-
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
-
44
-
-
0033759386
-
No deleterious mutations in the FOXJ1 (alias HFH-4) gene in patients with primary ciliary dyskinesia (PCD)
-
Maiti AK Bartoloni L Mitchison HM Meeks M Chung E Spiden S Gehrig C Rossier C DeLozier-Blanchet CD Blouin J Gardiner RM Antonarakis SE No deleterious mutations in the FOXJ1 (alias HFH-4) gene in patients with primary ciliary dyskinesia (PCD) Cytogenet Cell Genet 2000 90 119 22
-
(2000)
Cytogenet Cell Genet
, vol.90
, pp. 119-122
-
-
Maiti, A.K.1
Bartoloni, L.2
Mitchison, H.M.3
Meeks, M.4
Chung, E.5
Spiden, S.6
Gehrig, C.7
Rossier, C.8
DeLozier-Blanchet, C.D.9
Blouin, J.10
Gardiner, R.M.11
Antonarakis, S.E.12
-
47
-
-
0032433707
-
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
-
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
-
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
-
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
-
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
-
52
-
-
14344274965
-
Disruption of an inner arm dynein heavy chain gene results in asthenozoospermia and reduced ciliary beat frequency
-
Neesen J Kirschner R Ochs M Schmiedl A Habermann B Mueller C Holstein AF Nuesslein T Adham I Engel W Disruption of an inner arm dynein heavy chain gene results in asthenozoospermia and reduced ciliary beat frequency Hum Mol Genet 2001 10 1117 28
-
(2001)
Hum Mol Genet
, vol.10
, pp. 1117-1128
-
-
Neesen, J.1
Kirschner, R.2
Ochs, M.3
Schmiedl, A.4
Habermann, B.5
Mueller, C.6
Holstein, A.F.7
Nuesslein, T.8
Adham, I.9
Engel, W.10
-
53
-
-
0033555998
-
Discordant organ laterality in monozygotic twins with primary ciliary dyskinesia
-
Noone PG Bali D Carson JL Sannuti A Gipson CL Ostrowski LE Bromberg PA Boucher RC Knowles MR Discordant organ laterality in monozygotic twins with primary ciliary dyskinesia Am J Med Genet 1999 82 155 60
-
(1999)
Am J Med Genet
, vol.82
, pp. 155-160
-
-
Noone, P.G.1
Bali, D.2
Carson, J.L.3
Sannuti, A.4
Gipson, C.L.5
Ostrowski, L.E.6
Bromberg, P.A.7
Boucher, R.C.8
Knowles, M.R.9
-
54
-
-
0442313529
-
Primary ciliary dyskinesia: diagnostic and phenotypic features
-
Noone PG Leigh MW Sannuti A Minnix SL Carson JL Hazucha M Zariwala MA Knowles MR Primary ciliary dyskinesia: diagnostic and phenotypic features Am J Respir Crit Care Med 2004 169 459 67
-
(2004)
Am J Respir Crit Care Med
, vol.169
, pp. 459-467
-
-
Noone, P.G.1
Leigh, M.W.2
Sannuti, A.3
Minnix, S.L.4
Carson, J.L.5
Hazucha, M.6
Zariwala, M.A.7
Knowles, M.R.8
-
56
-
-
0036479029
-
Mutations in DNAH5 cause primary ciliary dyskinesia and randomization of left-right asymmetry
-
Olbrich H Haffner K Kispert A Volkel A Volz A Sasmaz G Reinhardt R Hennig S Lehrach H Konietzko N Zariwala M Noone PG Knowles M Mitchison HM Meeks M Chung EM Hildebrandt F Sudbrak R Omran H Mutations in DNAH5 cause primary ciliary dyskinesia and randomization of left-right asymmetry Nat Genet 2002 30 143 4
-
(2002)
Nat Genet
, vol.30
, pp. 143-144
-
-
Olbrich, H.1
Haffner, K.2
Kispert, A.3
Volkel, A.4
Volz, A.5
Sasmaz, G.6
Reinhardt, R.7
Hennig, S.8
Lehrach, H.9
Konietzko, N.10
Zariwala, M.11
Noone, P.G.12
Knowles, M.13
Mitchison, H.M.14
Meeks, M.15
Chung, E.M.16
Hildebrandt, F.17
Sudbrak, R.18
Omran, H.19
-
57
-
-
0019364354
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
69
-
-
0028014332
-
Smoke and viral infection cause cilia loss detectable by bronchoalveolar lavage cytology and dynein ELISA
-
Sisson JH Papi A Beckmann JD Leise KL Wisecarver J Brodersen BW Kelling CL Spurzem JR Rennard SI Smoke and viral infection cause cilia loss detectable by bronchoalveolar lavage cytology and dynein ELISA Am J Respir Crit Care Med 1994 149 205 13
-
(1994)
Am J Respir Crit Care Med
, vol.149
, pp. 205-213
-
-
Sisson, J.H.1
Papi, A.2
Beckmann, J.D.3
Leise, K.L.4
Wisecarver, J.5
Brodersen, B.W.6
Kelling, C.L.7
Spurzem, J.R.8
Rennard, S.I.9
-
71
-
-
33645464522
-
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
-
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
-
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
-
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
-
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
-
77
-
-
27444444705
-
Normal and cystic fibrosis airway surface liquid homeostasis: the effects of phasic shear stress and viral infections
-
Tarran R Button B Picher M Paradiso AM Ribeiro CM Lazarowski ER Zhang L Collins PL Pickles RJ Fredburg JJ Boucher RC Normal and cystic fibrosis airway surface liquid homeostasis: the effects of phasic shear stress and viral infections J Biol Chem 2005 280 35751 9
-
(2005)
J Biol Chem
, vol.280
, pp. 35751-35759
-
-
Tarran, R.1
Button, B.2
Picher, M.3
Paradiso, A.M.4
Ribeiro, C.M.5
Lazarowski, E.R.6
Zhang, L.7
Collins, P.L.8
Pickles, R.J.9
Fredburg, J.J.10
Boucher, R.C.11
-
79
-
-
33646138489
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
87
-
-
0036167851
-
Effects of reduced mucus oxygen concentration in airway Pseudomonas infections of cystic fibrosis patients
-
Worlitzsch D Tarran R Ulrich M Schwab U Cekici A Meyer KC Birrer P Bellon G Berger J Weiss T Botzenhart K Yankaskas JR Randell S Boucher RC Doring G Effects of reduced mucus oxygen concentration in airway Pseudomonas infections of cystic fibrosis patients J Clin Invest 2002 109 317 25
-
(2002)
J Clin Invest
, vol.109
, pp. 317-325
-
-
Worlitzsch, D.1
Tarran, R.2
Ulrich, M.3
Schwab, U.4
Cekici, A.5
Meyer, K.C.6
Birrer, P.7
Bellon, G.8
Berger, J.9
Weiss, T.10
Botzenhart, K.11
Yankaskas, J.R.12
Randell, S.13
Boucher, R.C.14
Doring, G.15
-
88
-
-
25144453883
-
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
-
89
-
-
33749843285
-
Mutations of DNAI1 in primary ciliary dyskinesia: evidence of founder effect in a common mutation
-
Zariwala MA Leigh MW Ceppa F Kennedy MP Horvath J Olbrich H Loges NT Duriez B Escudier E Mitchison HM Chodhari R Chung EM Morgan LM de Iongh RU Rutland J Pradal U Omran H Amselem S Knowles MR Mutations of DNAI1 in primary ciliary dyskinesia: evidence of founder effect in a common mutation Am J Respir Crit Care Med 2006 174 858 66
-
(2006)
Am J Respir Crit Care Med
, vol.174
, pp. 858-866
-
-
Zariwala, M.A.1
Leigh, M.W.2
Ceppa, F.3
Kennedy, M.P.4
Horvath, J.5
Olbrich, H.6
Loges, N.T.7
Duriez, B.8
Escudier, E.9
Mitchison, H.M.10
Chodhari, R.11
Chung, E.M.12
Morgan, L.M.13
de Iongh, R.U.14
Rutland, J.15
Pradal, U.16
Omran, H.17
Amselem, S.18
Knowles, M.R.19
-
90
-
-
1842451669
-
Investigation of the possible role of a novel gene, DPCD, in primary ciliary dyskinesia
-
Zariwala M O’Neal WK Noone PG Leigh MW Knowles MR Ostrowski LE Investigation of the possible role of a novel gene, DPCD, in primary ciliary dyskinesia Am J Respir Cell Mol Biol 2004 30 428 34
-
(2004)
Am J Respir Cell Mol Biol
, vol.30
, pp. 428-434
-
-
Zariwala, M.1
O’Neal, W.K.2
Noone, P.G.3
Leigh, M.W.4
Knowles, M.R.5
Ostrowski, L.E.6
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