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B. Schwartz, Craig Williams, P. Bernhardt (2008)
End game strategies towards the total synthesis of vibsanin E, 3-hydroxyvibsanin E, furanovibsanin A, and 3-O-methylfuranovibsanin ABeilstein Journal of Organic Chemistry, 4
M. Goutaki, Elisabeth Maurer, F. Halbeisen, I. Amirav, A. Barbato, L. Behan, M. Boon, C. Casaulta, A. Clément, S. Crowley, E. Haarman, C. Hogg, B. Karadag, C. Koerner-Rettberg, M. Leigh, M. Loebinger, H. Mazurek, L. Morgan, K. Nielsen, H. Omran, N. Schwerk, S. Scigliano, C. Werner, P. Yiallouros, Z. Zivković, J. Lucas, C. Kuehni (2017)
The international primary ciliary dyskinesia cohort (iPCD Cohort): methods and first resultsThe European Respiratory Journal, 49
G. Solomon, Richard Francis, K. Chu, S. Birket, George Gabriel, John Trombley, Kristi Lemke, N. Klena, Brett Turner, G. Tearney, C. Lo, S. Rowe (2017)
Assessment of ciliary phenotype in primary ciliary dyskinesia by micro-optical coherence tomography.JCI insight, 2 5
C. Banks, Laura Freeman, D. Cho, B. Woodworth (2018)
Acquired cystic fibrosis transmembrane conductance regulator dysfunctionWorld Journal of Otorhinolaryngology - Head and Neck Surgery, 4
A. Tilley, M. Walters, Renat Shaykhiev, R. Crystal (2015)
Cilia dysfunction in lung disease.Annual review of physiology, 77
G. Raghu, D. Weycker, J. Edelsberg, W. Bradford, G. Oster (2006)
Incidence and prevalence of idiopathic pulmonary fibrosis.American journal of respiratory and critical care medicine, 174 7
D. Gudis, N. Cohen (2010)
Cilia dysfunction.Otolaryngologic clinics of North America, 43 3
H. Lam, S. Cloonan, A. Bhashyam, J. Haspel, Anju Singh, J. Sathirapongsasuti, Morgan Cervo, H. Yao, Anna Chung, Kenji Mizumura, C. An, B. Shan, Jonathan Franks, K. Haley, C. Owen, Y. Tesfaigzi, G. Washko, John Quackenbush, E. Silverman, I. Rahman, Hong-pyo Kim, Ashfaq Mahmood, S. Biswal, S. Ryter, A. Choi (2013)
Histone deacetylase 6-mediated selective autophagy regulates COPD-associated cilia dysfunction.The Journal of clinical investigation, 123 12
D. Coultas, R. Zumwalt, W. Black, R. Sobonya (1994)
The epidemiology of interstitial lung diseases.American journal of respiratory and critical care medicine, 150 4
Vamsee Raju, V. Lin, Limbo Liu, C. McNicholas, Suman Karki, Peter Sloane, Liping Tang, P. Jackson, Wei Wang, L. Wilson, K. Macon, M. Mazur, J. Kappes, L. DeLucas, Stephen Barnes, K. Kirk, G. Tearney, S. Rowe (2017)
The Cystic Fibrosis Transmembrane Conductance Regulator Potentiator Ivacaftor Augments Mucociliary Clearance Abrogating Cystic Fibrosis Transmembrane Conductance Regulator Inhibition by Cigarette SmokeAmerican Journal of Respiratory Cell and Molecular Biology, 56
A. Shoemark (2017)
Applications of emerging transmission electron microscopy technology in PCD research and diagnosisUltrastructural Pathology, 41
Kangkang Song, J. Awata, D. Tritschler, R. Bower, G. Witman, M. Porter, D. Nicastro (2015)
In Situ Localization of N and C Termini of Subunits of the Flagellar Nexin-Dynein Regulatory Complex (N-DRC) Using SNAP Tag and Cryo-electron Tomography*The Journal of Biological Chemistry, 290
Shaoyan Zhang, Nicholas Smith, D. Schuster, Christopher Azbell, E. Sorscher, S. Rowe, B. Woodworth (2011)
Quercetin Increases Cystic Fibrosis Transmembrane Conductance Regulator–Mediated Chloride Transport and Ciliary Beat Frequency: Therapeutic Implications for Chronic RhinosinusitisAmerican Journal of Rhinology & Allergy, 25
Gregg Duncan, James Jung, A. Joseph, A. Thaxton, N. West, M. Boyle, J. Hanes, J. Suk (2016)
Microstructural alterations of sputum in cystic fibrosis lung disease.JCI insight, 1 18
K. Lechtreck, T. Gould, G. Witman (2013)
Flagellar central pair assembly in Chlamydomonas reinhardtiiCilia, 2
Karla Droguett, M. Ríos, D. Carreño, C. Navarrete, Christian Fuentes, M. Villalón, N. Barrera (2017)
An autocrine ATP release mechanism regulates basal ciliary activity in airway epitheliumThe Journal of Physiology, 595
A. Shapiro, Maureen Josephson, M. Rosenfeld, O. Yılmaz, S. Davis, D. Polineni, E. Guadagno, M. Leigh, V. Lavergne (2017)
Accuracy of Nasal Nitric Oxide Measurement as a Diagnostic Test for Primary Ciliary Dyskinesia. A Systematic Review and Meta‐analysisAnnals of the American Thoracic Society, 14
M. Leigh, M. Knowles (2017)
Assessment of Ciliary Beat Pattern: Variability in Healthy Control Subjects Has Implications for Use as Test for Primary Ciliary Dyskinesia.Chest, 151 5
Kiranya Tipirneni, D. Cho, D. Skinner, Shaoyan Zhang, Calvin Mackey, D. Lim, B. Woodworth (2017)
Characterization of primary rat nasal epithelial cultures in CFTR knockout rats as a model for CF sinus diseaseThe Laryngoscope, 127
A. Shoemark, C. Hogg (2012)
Electron tomography of respiratory ciliaThorax, 68
M. Silverman, M. Leroux (2009)
Intraflagellar transport and the generation of dynamic, structurally and functionally diverse cilia.Trends in cell biology, 19 7
N. Spassky, A. Meunier (2017)
The development and functions of multiciliated epitheliaNature Reviews Molecular Cell Biology, 18
(2002)
Copyright #ERS Journals Ltd 2003 European Respiratory Journal
T. Burgoyne, M. Dixon, P. Luther, C. Hogg, A. Shoemark (2012)
Generation of a three-dimensional ultrastructural model of human respiratory cilia.American journal of respiratory cell and molecular biology, 47 6
Jennifer Stubbs, Isao Oishi, J. Belmonte, C. Kintner (2008)
The Forkhead protein, FoxJ1, specifies node-like cilia in Xenopus and Zebrafish embryosNature genetics, 40
C. Kempeneers, Claire Seaton, M. Chilvers (2017)
Variation of Ciliary Beat Pattern in Three Different Beating Planes in Healthy SubjectsChest, 151
Robert Bloodgood (2010)
Sensory reception is an attribute of both primary cilia and motile ciliaJournal of Cell Science, 123
P Satir (1968)
Studies on cilia. III. Further studies on the cilium tip and a “sliding filament” model of ciliary motility, 39
S. Birket, K. Chu, Linbo Liu, Grace Houser, Bradford Diephuis, E. Wilsterman, Gregory Dierksen, M. Mazur, S. Shastry, Yao Li, J. Watson, Alexander Smith, Benjamin Schuster, J. Hanes, W. Grizzle, E. Sorscher, G. Tearney, S. Rowe (2014)
A functional anatomic defect of the cystic fibrosis airway.American journal of respiratory and critical care medicine, 190 4
M. Olm, J. Kögler, M. Macchione, A. Shoemark, P. Saldiva, J. Rodrigues (2011)
Primary ciliary dyskinesia: evaluation using cilia beat frequency assessment via spectral analysis of digital microscopy images.Journal of applied physiology, 111 1
G Raghu, HR Collard, JJ Egan, FJ Martinez, J Behr, KK Brown, TV Colby, JF Cordier, KR Flaherty, JA Lasky, DA Lynch, JH Ryu, JJ Swigris, AU Wells, J Ancochea, D Bouros, C Carvalho, U Costabel, M Ebina, DM Hansell, T Johkoh, DS Kim, TE King, Y Kondoh, J Myers, NL Müller, AG Nicholson, L Richeldi, M Selman, RF Dudden, BS Griss, SL Protzko, HJ Schünemann (2011)
An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management, 183
(2017)
Overexpression of Muc5b in the large airways causes delayed mucus transport in mice
M. Winey, J. Meehl, E. O'Toole, T. Giddings (2014)
Conventional transmission electron microscopyMolecular Biology of the Cell, 25
B. Button, R. Boucher (2008)
Role of mechanical stress in regulating airway surface hydration and mucus clearance ratesRespiratory Physiology & Neurobiology, 163
E. Vladar, J. Nayak, C. Milla, J. Axelrod (2016)
Airway epithelial homeostasis and planar cell polarity signaling depend on multiciliated cell differentiation.JCI insight, 1 13
M. Boon, I. Meyts, M. Proesmans, F. Vermeulen, M. Jorissen, K. Boeck (2014)
Diagnostic accuracy of nitric oxide measurements to detect primary ciliary dyskinesiaEuropean Journal of Clinical Investigation, 44
Alok Shah, Y. Ben-Shahar, T. Moninger, J. Kline, M. Welsh (2009)
Motile Cilia of Human Airway Epithelia Are ChemosensoryScience, 325
G. Mantovani, M. Pifferi, G. Vozzi (2010)
Automated software for analysis of ciliary beat frequency and metachronal wave orientation in primary ciliary dyskinesiaEuropean Archives of Oto-Rhino-Laryngology, 267
Jianfeng Lin, W. Yin, Maria Smith, Kangkang Song, M. Leigh, M. Zariwala, M. Knowles, L. Ostrowski, D. Nicastro (2014)
Cryo-electron tomography reveals ciliary defects underlying human RSPH1 primary ciliary dyskinesiaNature communications, 5
J. Papon, André Coste, F. Roudot-thoraval, M. Boucherat, G. Roger, A. Tamalet, Anne-Marie Vojtek, Serge Amselem, Estelle Escudier (2009)
A 20-year experience of electron microscopy in the diagnosis of primary ciliary dyskinesiaEuropean Respiratory Journal, 35
M. Chilvers, C. O’Callaghan (2000)
Analysis of ciliary beat pattern and beat frequency using digital high speed imaging: comparison with the photomultiplier and photodiode methodsThorax, 55
M. Turner, Elizabeth Matthes, A. Billet, Amy Ferguson, David Thomas, S. Randell, L. Ostrowski, Kathy Abbott-Banner, J. Hanrahan (2016)
The dual phosphodiesterase 3 and 4 inhibitor RPL554 stimulates CFTR and ciliary beating in primary cultures of bronchial epithelia.American journal of physiology. Lung cellular and molecular physiology, 310 1
SM Cloonan, HC Lam, SW Ryter, AM Choi (2014)
“Ciliophagy”: The consumption of cilia components by autophagy, 10
N. Todd, S. Atamas, I. Luzina, J. Galvin (2015)
Permanent alveolar collapse is the predominant mechanism in idiopathic pulmonary fibrosisExpert Review of Respiratory Medicine, 9
M. Demedts, J. Behr, R. Buhl, U. Costabel, R. Dekhuijzen, H. Jansen, W. Macnee, M. Thomeer, B. Wallaert, F. Laurent, A. Nicholson, E. Verbeken, J. Verschakelen, C. Flower, F. Capron, S. Petruzzelli, P. Vuyst, J. Bosch, E. Rodríguez-Becerra, G. Corvasce, I. Lankhorst, M. Sardina, M. Montanari (2005)
High-dose acetylcysteine in idiopathic pulmonary fibrosis.The New England journal of medicine, 353 21
Zhihua Chen, Hong-pyo Kim, F. Sciurba, Seon-Jin Lee, C. Feghali-Bostwick, D. Stolz, R. Dhir, R. Landreneau, M. Schuchert, S. Yousem, K. Nakahira, J. Pilewski, Janet Lee, Yingze Zhang, S. Ryter, A. Choi (2008)
Egr-1 Regulates Autophagy in Cigarette Smoke-Induced Chronic Obstructive Pulmonary DiseasePLoS ONE, 3
T. Nordgren, T. Wyatt, J. Sweeter, K. Bailey, J. Poole, A. Heires, J. Sisson, D. Romberger (2014)
Motile cilia harbor serum response factor as a mechanism of environment sensing and injury response in the airway.American journal of physiology. Lung cellular and molecular physiology, 306 9
B. Jacquet, Raul Salinas-Mondragon, Huixuan Liang, B. Therit, Justin Buie, M. Dykstra, K. Campbell, L. Ostrowski, S. Brody, H. Ghashghaei (2009)
FoxJ1-dependent gene expression is required for differentiation of radial glia into ependymal cells and a subset of astrocytes in the postnatal brainDevelopment, 136
J. Rosenbaum, G. Witman (2002)
Intraflagellar transportNature Reviews Molecular Cell Biology, 3
Clifford Courville, S. Raju, Bo Liu, F. Accurso, M. Dransfield, Steven Rowe (2015)
Recovery of Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction after Smoking Cessation.American journal of respiratory and critical care medicine, 192 12
P Satir (1963)
Studies on cilia. The fixation of the metachronal wave, 18
A. Yaghi, M. Dolovich (2016)
Airway Epithelial Cell Cilia and Obstructive Lung DiseaseCells, 5
S. Cloonan, H. Lam, S. Ryter, A. Choi (2014)
“Ciliophagy”Autophagy, 10
M. Leigh (2003)
Primary Ciliary DyskinesiaSEMINARS IN RESPIRATORY AND CRITICAL CARE MEDICINE, 24
A. Boland, Leifu Chang, D. Barford (2017)
The potential of cryo-electron microscopy for structure-based drug design.Essays in biochemistry, 61 5
P Satir (1965)
Studies on cilia. II. Examination of the distal region of the ciliary shaft and the role of the filaments in motility, 26
M. Leigh, T. Ferkol, S. Davis, Hye-Seung Lee, M. Rosenfeld, S. Dell, S. Sagel, C. Milla, K. Olivier, K. Sullivan, M. Zariwala, J. Pittman, A. Shapiro, J. Carson, J. Krischer, M. Hazucha, M. Knowles (2016)
Clinical Features and Associated Likelihood of Primary Ciliary Dyskinesia in Children and Adolescents.Annals of the American Thoracic Society, 13 8
Oberarzt Kartagener (1933)
Zur Pathogenese der BronchiektasienBeiträge zur Klinik der Tuberkulose und spezifischen Tuberkulose-Forschung, 83
P. Satir (1963)
STUDIES ON CILIAThe Journal of Cell Biology, 18
A. Onoufriadis, T. Paff, D. Antony, A. Shoemark, D. Micha, B. Kuyt, M. Schmidts, S. Petridi, J. Dankert-Roelse, E. Haarman, J. Daniels, Richard Emes, R. Wilson, C. Hogg, P. Scambler, E. Chung, G. Pals, H. Mitchison (2013)
Splice-site mutations in the axonemal outer dynein arm docking complex gene CCDC114 cause primary ciliary dyskinesia.American journal of human genetics, 92 1
Fay Newton, P. Lage, Somdatta Karak, D. Moore, M. Göpfert, A. Jarman (2012)
Forkhead Transcription Factor Fd3F Cooperates with Rfx to Regulate a Gene Expression Program for Mechanosensory Cilia SpecializationDevelopmental Cell, 22
S. Davis, T. Ferkol, M. Rosenfeld, Hye-Seung Lee, S. Dell, S. Sagel, C. Milla, M. Zariwala, J. Pittman, A. Shapiro, J. Carson, J. Krischer, M. Hazucha, M. Cooper, M. Knowles, M. Leigh (2015)
Clinical features of childhood primary ciliary dyskinesia by genotype and ultrastructural phenotype.American journal of respiratory and critical care medicine, 191 3
J. Raidt, J. Wallmeier, R. Hjeij, J. Onnebrink, P. Pennekamp, N. Loges, H. Olbrich, K. Häffner, G. Dougherty, H. Omran, C. Werner (2014)
Ciliary beat pattern and frequency in genetic variants of primary ciliary dyskinesiaEuropean Respiratory Journal, 44
A. Lansley, M. Sanderson (1999)
Regulation of airway ciliary activity by Ca2+: simultaneous measurement of beat frequency and intracellular Ca2+.Biophysical journal, 77 1
E. Nigg, J. Raff (2009)
Centrioles, Centrosomes, and Cilia in Health and DiseaseCell, 139
Bryan Lemieux, Jason Chen, Joseph Jing, Zhongping Chen, B. Wong (2015)
Measurement of ciliary beat frequency using Doppler optical coherence tomographyInternational Forum of Allergy & Rhinology, 5
Ximena Bustamante-Marin, L. Ostrowski (2017)
Cilia and Mucociliary Clearance.Cold Spring Harbor perspectives in biology, 9 4
Anh Innes, P. Woodruff, R. Ferrando, Samantha Donnelly, G. Dolganov, S. Lazarus, J. Fahy (2006)
Epithelial mucin stores are increased in the large airways of smokers with airflow obstruction.Chest, 130 4
J. Fahy, B. Dickey (2010)
Airway mucus function and dysfunction.The New England journal of medicine, 363 23
M. Leigh, J. Pittman, J. Carson, T. Ferkol, S. Dell, S. Davis, M. Knowles, M. Zariwala (2009)
Clinical and genetic aspects of primary ciliary dyskinesia/Kartagener syndromeGenetics in Medicine, 11
(2017)
First in human functional anatomic imaging of nasal airways using OCT
G. Raghu, K. Anstrom, T. King, J. Lasky, F. Martinez (2012)
Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis.The New England journal of medicine, 366 21
Biju Thomas, A. Rutman, C. O’Callaghan (2009)
Disrupted ciliated epithelium shows slower ciliary beat frequency and increased dyskinesiaEuropean Respiratory Journal, 34
S. Wiscombe, I. Forrest, A. Simpson (2013)
IPF: time for the (ciliary) beat generation?Thorax, 68
T. Ishikawa (2017)
Axoneme Structure from Motile Cilia.Cold Spring Harbor perspectives in biology, 9 1
J. Lucas, A. Burgess, H. Mitchison, E. Moya, M. Williamson, C. Hogg (2014)
Diagnosis and management of primary ciliary dyskinesiaArchives of Disease in Childhood, 99
Iram Haq, M. Gray, J. Garnett, C. Ward, M. Brodlie (2015)
Airway surface liquid homeostasis in cystic fibrosis: pathophysiology and therapeutic targetsThorax, 71
G. Solomon, S. Raju, M. Dransfield, S. Rowe (2016)
Therapeutic Approaches to Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction in Chronic Bronchitis.Annals of the American Thoracic Society, 13 Suppl 2
John McArdle1, Laurie Whittaker2 (2009)
Cystic FibrosisSemin Respir Crit Care Med, 30
Zvi Teff, Z. Priel, Levi Gheber (2007)
Forces applied by cilia measured on explants from mucociliary tissue.Biophysical journal, 92 5
H. Mitchison, A. Shoemark (2017)
Motile cilia defects in diseases other than primary ciliary dyskinesia: The contemporary diagnostic and research role for transmission electron microscopyUltrastructural Pathology, 41
N. Damseh, N. Quercia, Nisreen Rumman, S. Dell, R. Kim (2017)
Primary ciliary dyskinesia: mechanisms and managementThe Application of Clinical Genetics, 10
C. Bear, Canhui Li, N. Kartner, R. Bridges, T. Jensen, M. Ramjeesingh, J. Riordan (1992)
Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR)Cell, 68
I. Gibbons, A. Rowe (1965)
Dynein: A Protein with Adenosine Triphosphatase Activity from CiliaScience, 149
Justina Hessel, Jonna Heldrich, Jennifer Fuller, Michelle Staudt, Sharon Radisch, C. Hollmann, B. Harvey, R. Kaner, J. Salit, Jenny Yee-Levin, S. Sridhar, S. Pillai, Holly Hilton, G. Wolff, H. Bitter, S. Visvanathan, J. Fine, Christopher Stevenson, R. Crystal, A. Tilley (2014)
Intraflagellar Transport Gene Expression Associated with Short Cilia in Smoking and COPDPLoS ONE, 9
Catherine Oikonomou, G. Jensen (2017)
Cellular Electron Cryotomography: Toward Structural Biology In Situ.Annual review of biochemistry, 86
V. Mutyam, Emily Libby, N. Peng, D. Hadjiliadis, M. Bonk, G. Solomon, S. Rowe (2017)
Therapeutic benefit observed with the CFTR potentiator, ivacaftor, in a CF patient homozygous for the W1282X CFTR nonsense mutation.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 16 1
H. Ueno, K. Bui, T. Ishikawa, Y. Imai, Takami Yamaguchi, T. Ishikawa (2014)
Structure of dimeric axonemal dynein in cilia suggests an alternative mechanism of force generationCytoskeleton, 71
N. Kotov, D. Bates, Antonina Gizatullina, Bulat Gilaziev, R. Khairullin, Michael Chen, Michael Chen, I. Drozdov, Y. Umezawa, C. Hundhausen, A. Aleksandrov, Xing-Gang Yan, S. Spurgeon, C. Smales, N. Valeyev (2011)
Computational modelling elucidates the mechanism of ciliary regulation in health and diseaseBMC Systems Biology, 5
W. Stannard, M. Chilvers, A. Rutman, Christopher Williams, C. O’Callaghan (2010)
Diagnostic testing of patients suspected of primary ciliary dyskinesia.American journal of respiratory and critical care medicine, 181 4
M. Seibold, A. Wise, M. Speer, M. Steele, K. Brown, J. Loyd, T. Fingerlin, Weiming Zhang, G. Gudmundsson, S. Groshong, C. Evans, S. Garantziotis, K. Adler, B. Dickey, R. Bois, Ivana Yang, A. Herron, D. Kervitsky, Janet Talbert, C. Markin, Joungjoa Park, A. Crews, S. Slifer, S. Auerbach, Michelle Roy, Jia Lin, Corinne Hennessy, M. Schwarz, D. Schwartz (2011)
A Common MUC 5 B Promoter Polymorphism and Pulmonary Fibrosis
K. Murata, M. Wolf (2018)
Cryo-electron microscopy for structural analysis of dynamic biological macromolecules.Biochimica et biophysica acta. General subjects, 1862 2
Linbo Liu, K. Chu, Grace Houser, Bradford Diephuis, Yao Li, E. Wilsterman, S. Shastry, Gregory Dierksen, S. Birket, M. Mazur, Suzanne Byan-Parker, W. Grizzle, E. Sorscher, S. Rowe, G. Tearney (2013)
Method for Quantitative Study of Airway Functional Microanatomy Using Micro-Optical Coherence TomographyPLoS ONE, 8
J. Sisson, J. Stoner, B. Ammons, T. Wyatt (2003)
All‐digital image capture and whole‐field analysis of ciliary beat frequencyJournal of Microscopy, 211
C. Vogelmeier, G. Criner, F. Martinez, A. Anzueto, P. Barnes, J. Bourbeau, B. Celli, Rongchang Chen, M. Decramer, L. Fabbri, P. Frith, David M.G. Halpin, M. Varela, M. Nishimura, N. Roche, R. Rodríguez-Roisín, D. Sin, Dave Singh, R. Stockley, J. Vestbo, J. Wedzicha, A. Agustí (2017)
Global Strategy for the Diagnosis, Management, and Prevention of Chronic Obstructive Lung Disease 2017 Report: GOLD Executive SummaryEuropean Respiratory Journal, 49
M. Turner, M. Jerrett, C. Pope, D. Krewski, S. Gapstur, W. Diver, B. Beckerman, J. Marshall, Jason Su, Dan Crouse, R. Burnett (2016)
Long-Term Ozone Exposure and Mortality in a Large Prospective Study.American journal of respiratory and critical care medicine, 193 10
J. Lucas, A. Barbato, Samuel Collins, M. Goutaki, L. Behan, D. Caudri, S. Dell, E. Eber, E. Escudier, R. Hirst, C. Hogg, M. Jorissen, P. Latzin, M. Legendre, M. Leigh, F. Midulla, K. Nielsen, H. Omran, J. Papon, P. Pohunek, Beatrice Redfern, D. Rigau, B. Rindlisbacher, F. Santamaria, A. Shoemark, D. Snijders, T. Tonia, A. Titieni, W. Walker, C. Werner, A. Bush, C. Kuehni (2017)
European Respiratory Society guidelines for the diagnosis of primary ciliary dyskinesiaEuropean Respiratory Journal, 49
V. Prulière-Escabasse, A. Coste, P. Chauvin, B. Fauroux, A. Tamalet, E. Garabédian, E. Escudier, G. Roger (2010)
Otologic features in children with primary ciliary dyskinesia.Archives of otolaryngology--head & neck surgery, 136 11
M. Sanderson, E. Dirksen (1985)
A versatile and quantitative computer-assisted photoelectronic technique used for the analysis of ciliary beat cycles.Cell motility, 5 4
S. Ishijima (1995)
High-speed video microscopy of flagella and cilia.Methods in cell biology, 47
M. Knowles, R. Boucher (2002)
Mucus clearance as a primary innate defense mechanism for mammalian airways.The Journal of clinical investigation, 109 5
S. Rosselot (2014)
Idiopathic pulmonary fibrosis.Nursing standard (Royal College of Nursing (Great Britain) : 1987), 28 52
A. Shapiro, M. Zariwala, T. Ferkol, S. Davis, S. Sagel, S. Dell, M. Rosenfeld, K. Olivier, C. Milla, S. Daniel, A. Kimple, M. Manion, M. Knowles, M. Leigh (2015)
Diagnosis, monitoring, and treatment of primary ciliary dyskinesia: PCD foundation consensus recommendations based on state of the art reviewPediatric Pulmonology, 51
S. Choksi, G. Lauter, P. Swoboda, Sudipto Roy (2014)
Switching on cilia: transcriptional networks regulating ciliogenesisDevelopment, 141
D. Fawcett (1954)
The study of epithelial cilia and sperm flagella with the electron microscope †The Laryngoscope, 64
J. Gribbin, R. Hubbard, I. Jeune, Chris Smith, J. West, Laila (2006)
Incidence and mortality of idiopathic pulmonary fibrosis and sarcoidosis in the UKThorax, 61
M. Knowles, L. Ostrowski, M. Leigh, P. Sears, S. Davis, Whitney Wolf, M. Hazucha, J. Carson, K. Olivier, S. Sagel, M. Rosenfeld, T. Ferkol, S. Dell, C. Milla, S. Randell, W. Yin, A. Sannuti, Hilda Metjian, P. Noone, Peter Noone, C. Olson, M. Patrone, H. Dang, Hye-Seung Lee, T. Hurd, H. Gee, E. Otto, J. Halbritter, Stefan Kohl, Martin Kircher, J. Krischer, M. Bamshad, D. Nickerson, F. Hildebrandt, J. Shendure, M. Zariwala (2014)
Mutations in RSPH1 cause primary ciliary dyskinesia with a unique clinical and ciliary phenotype.American journal of respiratory and critical care medicine, 189 6
G. Solomon, H. Hathorne, Bo Liu, S. Raju, G. Reeves, Edward Acosta, M. Dransfield, Steven Rowe (2016)
Pilot evaluation of ivacaftor for chronic bronchitis.The Lancet. Respiratory medicine, 4 6
B. Button, Seiko Okada, C. Frederick, W. Thelin, R. Boucher (2013)
Mechanosensitive ATP Release Maintains Proper Mucus Hydration of AirwaysScience Signaling, 6
M. Seibold, Russell Smith, Cydney Urbanek, S. Groshong, G. Cosgrove, K. Brown, M. Schwarz, D. Schwartz, S. Reynolds (2013)
The Idiopathic Pulmonary Fibrosis Honeycomb Cyst Contains A Mucocilary Pseudostratified EpitheliumPLoS ONE, 8
Anne Moore, E. Escudier, G. Roger, A. Tamalet, B. Pelosse, S. Marlin, A. Clément, M. Geremek, B. Delaisi, A. Bridoux, A. Coste, M. Witt, B. Duriez, S. Amselem (2005)
RPGR is mutated in patients with a complex X linked phenotype combining primary ciliary dyskinesia and retinitis pigmentosaJournal of Medical Genetics, 43
S. Dimova, F. Maes, M. Brewster, M. Jorissen, M. Noppe, P. Augustijns (2005)
High‐speed digital imaging method for ciliary beat frequency measurementJournal of Pharmacy and Pharmacology, 57
S. Christensen, I. Veland, A. Schwab, M. Cammer, P. Satir (2013)
Analysis of primary cilia in directional cell migration in fibroblasts.Methods in enzymology, 525
R. Dresdner, L. Wong (1985)
Measurement of ciliary beat frequency using high-speed video microscopy.ISA transactions, 24 1
Joseph Jing, Jason Chen, Li-dek Chou, B. Wong, Zhongping Chen (2017)
Visualization and Detection of Ciliary Beating Pattern and Frequency in the Upper Airway using Phase Resolved Doppler Optical Coherence TomographyScientific Reports, 7
L. Didon, Rachel Zwick, I. Chao, M. Walters, Rui Wang, N. Hackett, R. Crystal (2013)
RFX3 Modulation of FOXJ1 regulation of cilia genes in the human airway epitheliumRespiratory Research, 14
Xianwen Yu, C. Ng, Hermann Habacher, Sudipto Roy (2008)
Foxj1 transcription factors are master regulators of the motile ciliogenic programNature Genetics, 40
S. Raju, Hyunki Kim, S. Byzek, Li Tang, John Trombley, P. Jackson, L. Rasmussen, Wells Jn, Emily Libby, Erik Dohm, Lindy Winter, Sharon Samuel, Kurt Zinn, J. Blalock, T. Schoeb, M. Dransfield, Steven Rowe (2016)
A ferret model of COPD-related chronic bronchitis.JCI insight, 1 15
F. Garcia-Gonzalo, Jeremy Reiter (2012)
Scoring a backstage pass: Mechanisms of ciliogenesis and ciliary accessThe Journal of Cell Biology, 197
W. Marshall, J. Rosenbaum (2001)
Intraflagellar transport balances continuous turnover of outer doublet microtubulesThe Journal of Cell Biology, 155
A. Horani, S. Brody, T. Ferkol (2013)
Picking up speed: advances in the genetics of primary ciliary dyskinesiaPediatric research, 75
S. Raju, P. Jackson, Clifford Courville, C. McNicholas, Peter Sloane, G. Sabbatini, Sherry Tidwell, L. Tang, Bo Liu, J. Fortenberry, Caleb Jones, J. Boydston, J. Clancy, L. Bowen, F. Accurso, J. Blalock, M. Dransfield, S. Rowe (2013)
Cigarette smoke induces systemic defects in cystic fibrosis transmembrane conductance regulator function.American journal of respiratory and critical care medicine, 188 11
L. Zein, H. Omran, P. Bouvagnet (2003)
Lateralization defects and ciliary dyskinesia: lessons from algae.Trends in genetics : TIG, 19 3
Toshiyuki Oda, M. Kikkawa (2013)
Novel structural labeling method using cryo-electron tomography and biotin-streptavidin system.Journal of structural biology, 183 3
M. Dransfield, A. Wilhelm, Brian Flanagan, Clifford Courville, Sherry Tidwell, S. Raju, A. Gaggar, C. Steele, L. Tang, Bo Liu, S. Rowe (2013)
Acquired cystic fibrosis transmembrane conductance regulator dysfunction in the lower airways in COPD.Chest, 144 2
G. Ripandelli, A. Coppè, Antonella Capaldo, M. Stirpe (1998)
Optical coherence tomography.Seminars in ophthalmology, 13 4
Linbo Liu, J. Gardecki, S. Nadkarni, J. Toussaint, Y. Yagi, B. Bouma, G. Tearney (2011)
Imaging the Subcellular Structure of Human Coronary Atherosclerosis Using 1-μm Resolution Optical Coherence Tomography (μOCT)Nature medicine, 17
G. Piatti, U. Ambrosetti, P. Santus, L. Allegra (2005)
Effects of salmeterol on cilia and mucus in COPD and pneumonia patients.Pharmacological research, 51 2
SE Birket, KK Chu, GH Houser, L Liu, CM Fernandez, GM Solomon, V Lin, S Shastry, M Mazur, PA Sloane, J Hanes, WE Grizzle, EJ Sorscher, GJ Tearney, SM Rowe (2016)
Combination therapy with cystic fibrosis transmembrane conductance regulator modulators augment the airway functional microanatomy, 310
T. Kon, Kenji Imamula, Anthony Roberts, R. Ohkura, Peter Knight, I. Gibbons, S. Burgess, K. Sutoh (2009)
Helix sliding in the stalk coiled coil of dynein couples ATPase and microtubule bindingNature structural & molecular biology, 16
C. Smith, J. Djakow, R. Free, Petr Djakow, Rana Lonnen, Gwyneth Williams, P. Pohunek, R. Hirst, A. Easton, P. Andrew, C. O’Callaghan (2012)
ciliaFA: a research tool for automated, high-throughput measurement of ciliary beat frequency using freely available softwareCilia, 1
B. Button, Liheng Cai, C. Ehre, M. Kesimer, D. Hill, J. Sheehan, R. Boucher, M. Rubinstein (2012)
A Periciliary Brush Promotes the Lung Health by Separating the Mucus Layer from Airway EpitheliaScience, 337
Rasagnya Viswanadha, W. Sale, M. Porter (2017)
Ciliary Motility: Regulation of Axonemal Dynein Motors.Cold Spring Harbor perspectives in biology, 9 8
PG Noone, MW Leigh, A Sannuti, SL Minnix, JL Carson, M Hazucha, MA Zariwala, MR Knowles (2004)
Primary ciliary dyskinesia: diagnostic and phenotypic features, 169
D. Narayan, S. Krishnan, M. Upender, T. Ravikumar, M. Mahoney, T. Dolan, A. Teebi, G. Haddad (1994)
Unusual inheritance of primary ciliary dyskinesia (Kartagener's syndrome).Journal of Medical Genetics, 31
(2016)
Metachrony markedly accelerates mucociliary transport in a Ca2ϩ and cAMP-dependent fashion. A79. Lung imaging: CT and beyond
S. Raju, G. Solomon, M. Dransfield, S. Rowe (2016)
Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction in Chronic Bronchitis and Other Diseases of Mucus Clearance.Clinics in chest medicine, 37 1
L Liu, JA Gardecki, SK Nadkarni, JD Toussaint, Y Yagi, BE Bouma, GJ Tearney (2011)
Imaging the subcellular structure of human coronary atherosclerosis using micro-optical coherence tomography, 17
N. Terzikhan, K. Verhamme, A. Hofman, B. Stricker, G. Brusselle, L. Lahousse (2016)
Prevalence and incidence of COPD in smokers and non-smokers: the Rotterdam StudyEuropean Journal of Epidemiology, 31
V. Mirra, C. Werner, F. Santamaria (2017)
Primary Ciliary Dyskinesia: An Update on Clinical Aspects, Genetics, Diagnosis, and Future Treatment StrategiesFrontiers in Pediatrics, 5
R Tarran, B Button, M Picher, AM Paradiso, CM Ribeiro, ER Lazarowski, L Zhang, PL Collins, RJ Pickles, JJ Fredberg, RC Boucher (2005)
Normal and cystic fibrosis airway surface liquid homeostasis. The effects of phasic shear stress and viral infections, 280
M. Hoegger, A. Fischer, James McMenimen, L. Ostedgaard, A. Tucker, Maged Awadalla, T. Moninger, A. Michalski, E. Hoffman, J. Zabner, D. Stoltz, M. Welsh (2014)
Impaired mucus detachment disrupts mucociliary transport in a piglet model of cystic fibrosisScience, 345
Richard Francis, C. Lo (2013)
Ex vivo method for high resolution imaging of cilia motility in rodent airway epithelia.Journal of visualized experiments : JoVE, 78
P. Satir (1995)
Landmarks in cilia research from Leeuwenhoek to us.Cell motility and the cytoskeleton, 32 2
R. Tarran, B. Button, M. Picher, A. Paradiso, C. Ribeiro, E. Lazarowski, Liqun Zhang, P. Collins, R. Pickles, J. Fredberg, R. Boucher (2005)
Normal and Cystic Fibrosis Airway Surface Liquid HomeostasisJournal of Biological Chemistry, 280
D. Devenport (2014)
The cell biology of planar cell polarityThe Journal of Cell Biology, 207
M. Knowles, L. Daniels, S. Davis, M. Zariwala, M. Leigh (2013)
Primary ciliary dyskinesia. Recent advances in diagnostics, genetics, and characterization of clinical disease.American journal of respiratory and critical care medicine, 188 8
WF Marshall, JL Rosenbaum (2001)
Intraflagellar transport balances continuous turnover of outer doublet microtubules: implications for flagellar length control, 155
A. Henderson, C. Ehre, B. Button, L. Abdullah, Liheng Cai, M. Leigh, Genevieve DeMaria, H. Matsui, S. Donaldson, C. Davis, J. Sheehan, R. Boucher, M. Kesimer (2014)
Cystic fibrosis airway secretions exhibit mucin hyperconcentration and increased osmotic pressure.The Journal of clinical investigation, 124 7
T Wodehouse, SA Kharitonov, IS Mackay, PJ Barnes, R Wilson, PJ Cole (2003)
Nasal nitric oxide measurements for the screening of primary ciliary dyskinesia, 21
Bryant Conger, Shaoyan Zhang, D. Skinner, S. Hicks, E. Sorscher, S. Rowe, B. Woodworth (2013)
Comparison of cystic fibrosis transmembrane conductance regulator (CFTR) and ciliary beat frequency activation by the CFTR Modulators Genistein, VRT-532, and UCCF-152 in primary sinonasal epithelial cultures.JAMA otolaryngology-- head & neck surgery, 139 8
S. Brody, Xiu‐Hua Yan, Mary Wuerffel, Sheng-Kwei Song, S. Shapiro (2000)
Ciliogenesis and left-right axis defects in forkhead factor HFH-4-null mice.American journal of respiratory cell and molecular biology, 23 1
Alok Shah, S. Farmen, T. Moninger, T. Businga, Michael Andrews, Kevin Bugge, C. Searby, D. Nishimura, K. Brogden, J. Kline, V. Sheffield, M. Welsh (2008)
Loss of Bardet–Biedl syndrome proteins alters the morphology and function of motile cilia in airway epitheliaProceedings of the National Academy of Sciences, 105
Christopher O'Callaghan, Phil Chetcuti, Eduardo Moya (2009)
High prevalence of primary ciliary dyskinesia in a British Asian populationArchives of Disease in Childhood, 95
M. Leigh, M. Zariwala, M. Knowles (2009)
Primary ciliary dyskinesia: improving the diagnostic approachCurrent Opinion in Pediatrics, 21
P. Avasthi, W. Marshall (2012)
Stages of ciliogenesis and regulation of ciliary length.Differentiation; research in biological diversity, 83 2
C. Vogelmeier, G. Criner, F. Martinez, A. Anzueto, P. Barnes, J. Bourbeau, B. Celli, Rongchang Chen, M. Decramer, L. Fabbri, P. Frith, D. Halpin, M. Varela, M. Nishimura, N. Roche, R. Rodríguez-Roisín, D. Sin, Dave Singh, R. Stockley, J. Vestbo, J. Wedzicha, A. Agustí (2017)
Global Strategy for the Diagnosis, Management and Prevention of Chronic Obstructive Lung Disease 2017 ReportRespirology, 22
B. Afzelius (1976)
A human syndrome caused by immotile cilia.Science, 193 4250
Shannon Quinn, Shannon Quinn, M. Zahid, J. Durkin, Richard Francis, C. Lo, C. Chennubhotla (2015)
Automated identification of abnormal respiratory ciliary motion in nasal biopsiesScience Translational Medicine, 7
M. Salathe (2007)
Regulation of mammalian ciliary beating.Annual review of physiology, 69
D. Worlitzsch, R. Tarran, M. Ulrich, U. Schwab, Aynur Cekici, K. Meyer, P. Birrer, G. Bellon, J. Berger, T. Weiss, K. Botzenhart, J. Yankaskas, S. Randell, R. Boucher, G. Döring (2002)
Effects of reduced mucus oxygen concentration in airway Pseudomonas infections of cystic fibrosis patients.The Journal of clinical investigation, 109 3
P. Satir (2017)
CILIA: before and afterCilia, 6
Jianfeng Lin, K. Okada, Milen Raytchev, Maria Smith, D. Nicastro (2014)
Structural mechanism of the dynein powerstrokeNature cell biology, 16
Dongyao Cui, K. Chu, Biwei Yin, Tim Ford, C. Hyun, H. Leung, J. Gardecki, G. Solomon, S. Birket, Linbo Liu, S. Rowe, G. Tearney (2017)
Flexible, high-resolution micro-optical coherence tomography endobronchial probe toward in vivo imaging of cilia.Optics letters, 42 4
J. Hurst, T. Wilkinson, W. Perera, G. Donaldson, J. Wedzicha (2005)
Relationships among bacteria, upper airway, lower airway, and systemic inflammation in COPD.Chest, 127 4
A. Horani, T. Ferkol, S. Dutcher, S. Brody (2016)
Genetics and biology of primary ciliary dyskinesia.Paediatric respiratory reviews, 18
C. Kuehni, J. Lucas (2016)
Toward an Earlier Diagnosis of Primary Ciliary Dyskinesia. Which Patients Should Undergo Detailed Diagnostic Testing?Annals of the American Thoracic Society, 13 8
M. Seibold, A. Wise, M. Speer, M. Steele, K. Brown, J. Loyd, T. Fingerlin, Weiming Zhang, G. Gudmundsson, S. Groshong, C. Evans, S. Garantziotis, K. Adler, B. Dickey, R. Bois, Ivana Yang, A. Herron, D. Kervitsky, Janet Talbert, C. Markin, Joungjoa Park, A. Crews, S. Slifer, S. Auerbach, Michelle Roy, Jia Lin, Corinne Hennessy, M. Schwarz, D. Schwartz (2011)
A common MUC5B promoter polymorphism and pulmonary fibrosis.The New England journal of medicine, 364 16
P. Kouis, P. Yiallouros, N. Middleton, J. Evans, K. Kyriacou, S. Papatheodorou (2017)
Prevalence of primary ciliary dyskinesia in consecutive referrals of suspect cases and the transmission electron microscopy detection rate: a systematic review and meta-analysisPediatric Research, 81
A. Rab, S. Rowe, S. Raju, Z. Bebok, S. Matalon, J. Collawn (2013)
Cigarette smoke and CFTR: implications in the pathogenesis of COPD.American journal of physiology. Lung cellular and molecular physiology, 305 8
D. Hughes (2008)
Primary ciliary dyskinesia.Paediatrics & child health, 13 8
S. Vij, J. Rink, Hao Ho, D. Babu, M. Eitel, V. Narasimhan, Varnesh Tiku, J. Westbrook, B. Schierwater, Sudipto Roy (2012)
Evolutionarily Ancient Association of the FoxJ1 Transcription Factor with the Motile Ciliogenic ProgramPLoS Genetics, 8
A. Tarasiuk, M. Bar-Shimon, L. Gheber, A. Korngreen, Y. Grossman, Z. Priel (1995)
Extracellular ATP induces hyperpolarization and motility stimulation of ciliary cells.Biophysical journal, 68 3
J. Hogg, F. Chu, Soraya Utokaparch, R. Woods, W. Elliott, L. Buzatu, R. Cherniack, R. Rogers, F. Sciurba, H. Coxson, P. Paré (2004)
The nature of small-airway obstruction in chronic obstructive pulmonary disease.The New England journal of medicine, 350 26
H. Matsui, B. Grubb, R. Tarran, S. Randell, J. Gatzy, Chelsea Davis, R. Boucher (1998)
Evidence for Periciliary Liquid Layer Depletion, Not Abnormal Ion Composition, in the Pathogenesis of Cystic Fibrosis Airways DiseaseCell, 95
GM Solomon, L Hancock, C Hennessy, JL Casey, KB Turner, Z Liu, AB Carter, C Evans, DA Schwartz, SM Rowe (2017)
Overexpression of Muc5b in the large airways causes delayed mucus transport in mice. D21. Immune pathways in acute lung injury and fibrosis, 195
K. Oami, M. Takahashi (2002)
Identification of the Ca2+ Conductance Responsible for K+-induced Backward Swimming in Paramecium caudatumThe Journal of Membrane Biology, 190
T. Ishikawa (2013)
3D structure of eukaryotic flagella/cilia by cryo-electron tomographyBiophysics, 9
G. Raghu, H. Collard, J. Egan, F. Martinez, J. Behr, K. Brown, T. Colby, Jean-Franc, ois Cordier, K. Flaherty, J. Lasky, D. Lynch, J. Ryu, J. Swigris, A. Wells, J. Ancochea, D. Bouros, Carlos Carvalho, U. Costabel, M. Ebina, D. Hansell, T. Johkoh, D. Kim, T. King, Y. Kondoh, J. Myers, N. Müller, A. Nicholson, L. Richeldi, M. Selman, R. Dudden, Barbara Griss, Shandra Protzko, H. Schünemann (2011)
American Thoracic Society Documents An Official ATS / ERS / JRS / ALAT Statement : Idiopathic Pulmonary Fibrosis : Evidence-based Guidelines for Diagnosis and Management
D. Visscher, J. Myers (2006)
Histologic spectrum of idiopathic interstitial pneumonias.Proceedings of the American Thoracic Society, 3 4
T. Weiss, L. Gheber, V. Shoshan-Barmatz, Z. Priel (1992)
Possible mechanism of ciliary stimulation by extracellular ATP: Involvement of calcium-dependent potassium channels and exogenous Ca2+The Journal of Membrane Biology, 127
T. Ishikawa (2015)
Cryo-electron tomography of motile cilia and flagellaCilia, 4
S. Birket, K. Chu, Grace Houser, Linbo Liu, C. Fernandez, G. Solomon, V. Lin, S. Shastry, M. Mazur, Peter Sloane, J. Hanes, W. Grizzle, E. Sorscher, G. Tearney, S. Rowe (2016)
Ion Channels and Transporters in Lung Function and Disease Combination therapy with cystic fibrosis transmembrane conductance regulator modulators augment the airway functional microanatomy
Dennis Lutz, A. Gazdhar, E. Lopez-Rodriguez, C. Ruppert, P. Mahavadi, A. Günther, W. Klepetko, J. Bates, Bradford Smith, T. Geiser, M. Ochs, L. Knudsen (2015)
Alveolar derecruitment and collapse induration as crucial mechanisms in lung injury and fibrosis.American journal of respiratory cell and molecular biology, 52 2
G. Solomon, Lianwu Fu, S. Rowe, J. Collawn (2017)
The therapeutic potential of CFTR modulators for COPD and other airway diseasesCurrent Opinion in Pharmacology, 34
Joo-Yong Lee, H. Koga, Y. Kawaguchi, W. Tang, E. Wong, Ya‐sheng Gao, U. Pandey, Susmita Kaushik, Emily Tresse, Jianrong Lu, J. Taylor, A. Cuervo, T. Yao (2010)
HDAC6 controls autophagosome maturation essential for ubiquitin‐selective quality‐control autophagyThe EMBO Journal, 29
(2017)
Poster Session Abstracts.Pediatric pulmonology, 52 S47
Ivana Yang, L. Burch, M. Steele, J. Savov, J. Hollingsworth, Erin Mcelvania-Tekippe, K. Berman, M. Speer, T. Sporn, K. Brown, M. Schwarz, D. Schwartz (2007)
Gene expression profiling of familial and sporadic interstitial pneumonia.American journal of respiratory and critical care medicine, 175 1
Linbo Liu, S. Shastry, Suzanne Byan-Parker, Grace Houser, Kengyeh Chu, S. Birket, C. Fernandez, J. Gardecki, W. Grizzle, E. Wilsterman, E. Sorscher, S. Rowe, G. Tearney (2014)
An autoregulatory mechanism governing mucociliary transport is sensitive to mucus load.American journal of respiratory cell and molecular biology, 51 4
M. Jakus, C. Hall (1946)
Electron microscope observations of the trichocysts and cilia of Paramecium.The Biological bulletin, 91 02
M. Dixon, A. Shoemark (2017)
Secondary defects detected by transmission electron microscopy in primary ciliary dyskinesia diagnosticsUltrastructural Pathology, 41
Toshiyuki Oda (2017)
Three-dimensional structural labeling microscopy of cilia and flagella.Microscopy, 66 4
K. Chu, Carolin Unglert, Tim Ford, Dongyao Cui, R. Carruth, Kanwarpal Singh, Linbo Liu, S. Birket, G. Solomon, S. Rowe, G. Tearney (2016)
In vivo imaging of airway cilia and mucus clearance with micro-optical coherence tomography.Biomedical optics express, 7 7
J. Fujimoto, C. Pitris, C. Pitris, S. Boppart, S. Boppart, M. Brezinski (2000)
Optical coherence tomography: an emerging technology for biomedical imaging and optical biopsy.Neoplasia, 2 1-2
A. Yaghi, A. Zaman, G. Cox, M. Dolovich (2012)
Ciliary beating is depressed in nasal cilia from chronic obstructive pulmonary disease subjects.Respiratory medicine, 106 8
F. Goor, Sabine Hadida, P. Grootenhuis, B. Burton, Dong Cao, Tim Neuberger, Amanda Turnbull, Ashvani Singh, J. Joubran, Anna Hazlewood, Jinglan Zhou, Jason Mccartney, Vijayalaksmi Arumugam, C. Decker, Jennifer Yang, C. Young, E. Olson, Jeffery Wine, R. Frizzell, M. Ashlock, P. Negulescu (2009)
Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770Proceedings of the National Academy of Sciences, 106
Michael Sanderson, Andrew Charles, E. Dirksen (1990)
Mechanical stimulation and intercellular communication increases intracellular Ca2+ in epithelial cells.Cell regulation, 1 8
R. Jain, J. Pan, James Driscoll, J. Wisner, T. Huang, S. Gunsten, Y. You, S. Brody (2010)
Temporal relationship between primary and motile ciliogenesis in airway epithelial cells.American journal of respiratory cell and molecular biology, 43 6
R. Jain, Cylen Javidan-Nejad, J. Alexander-Brett, A. Horani, Michele Cabellon, M. Walter, S. Brody (2012)
Sensory functions of motile cilia and implication for bronchiectasis.Frontiers in bioscience, 4
A. Barbato, T. Frischer, C. Kuehni, D. Snijders, Inês Azevedo, G. Baktai, L. Bartoloni, E. Eber, A. Escribano, E. Haarman, B. Hesselmar, C. Hogg, M. Jorissen, J. Lucas, K. Nielsen, C. O’Callaghan, H. Omran, P. Pohunek, M. Strippoli, A. Bush (2009)
Primary ciliary dyskinesia: a consensus statement on diagnostic and treatment approaches in childrenEuropean Respiratory Journal, 34
Shaoyan Zhang, D. Skinner, S. Hicks, M. Bevensee, E. Sorscher, A. Lazrak, S. Matalon, C. McNicholas, B. Woodworth (2014)
Sinupret Activates CFTR and TMEM16A-Dependent Transepithelial Chloride Transport and Improves Indicators of Mucociliary ClearancePLoS ONE, 9
E. Ziętkiewicz, Zuzanna Bukowy-Bieryllo, K. Voelkel, Barbara Klimek, H. Dmeńska, A. Pogorzelski, A. Sulikowska-Rowińska, E. Rutkiewicz, M. Witt (2012)
Mutations in Radial Spoke Head Genes and Ultrastructural Cilia Defects in East-European Cohort of Primary Ciliary Dyskinesia PatientsPLoS ONE, 7
C. Kuehni, T. Frischer, M. Strippoli, Elisabeth Maurer, A. Bush, K. Nielsen, A. Escribano, J. Lucas, P. Yiallouros, H. Omran, E. Eber, C. O’Callaghan, D. Snijders, A. Barbato (2010)
Factors influencing age at diagnosis of primary ciliary dyskinesia in European childrenEuropean Respiratory Journal, 36
A. Schmid, Zoltan Sutto, N. Schmid, Lisa Novak, P. Ivonnet, G. Horvath, Gregory Conner, N. Fregien, M. Salathe (2010)
Decreased Soluble Adenylyl Cyclase Activity in Cystic Fibrosis Is Related to Defective Apical Bicarbonate Exchange and Affects Ciliary Beat Frequency Regulation*The Journal of Biological Chemistry, 285
The respiratory tract is lined with multiciliated epithelial cells that function to move mucus and trapped particles via the mucociliary transport apparatus. Genetic and acquired ciliopathies result in diminished mucociliary clearance, contributing to disease pathogenesis. Recent innovations in imaging technology have advanced our understanding of ciliary motion in health and disease states. Application of imaging modalities including transmission electron microscopy, high-speed video microscopy, and micron-optical coherence tomography could improve diagnostics and be applied for precision medicine. In this review, we provide an overview of ciliary motion, imaging modalities, and ciliopathic diseases of the respiratory system including primary ciliary dyskinesia, cystic fibrosis, chronic obstructive pulmonary disease, and idiopathic pulmonary fibrosis.
AJP - Lung Cellular and Molecular Physiology – The American Physiological Society
Published: Jun 1, 2018
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