Mucus clearance as a primary innate defense mechanism for mammalian airways MR Knowles, RC Boucher The Journal of clinical investigation 109 (5), 571-577, 2002 | 1656 | 2002 |
Relation between mutations of the cystic fibrosis gene and idiopathic pancreatitis JA Cohn, KJ Friedman, PG Noone, MR Knowles, LM Silverman, ... New England Journal of Medicine 339 (10), 653-658, 1998 | 1153 | 1998 |
Primary ciliary dyskinesia: diagnostic and phenotypic features PG Noone, MW Leigh, A Sannuti, SL Minnix, JL Carson, M Hazucha, ... American journal of respiratory and critical care medicine 169 (4), 459-467, 2004 | 911 | 2004 |
Consensus on the use and interpretation of cystic fibrosis mutation analysis in clinical practice C Castellani, H Cuppens, M Macek Jr, JJ Cassiman, E Kerem, P Durie, ... Journal of cystic fibrosis 7 (3), 179-196, 2008 | 867 | 2008 |
Mucus clearance and lung function in cystic fibrosis with hypertonic saline SH Donaldson, WD Bennett, KL Zeman, MR Knowles, R Tarran, ... New England Journal of Medicine 354 (3), 241-250, 2006 | 857 | 2006 |
Increased bioelectric potential difference across respiratory epithelia in cystic fibrosis M Knowles, J Gatzy, R Boucher New England Journal of Medicine 305 (25), 1489-1495, 1981 | 763 | 1981 |
Nontuberculous mycobacteria: I: multicenter prevalence study in cystic fibrosis KN Olivier, DJ Weber, RJ Wallace Jr, AR Faiz, JH Lee, Y Zhang, ... American journal of respiratory and critical care medicine 167 (6), 828-834, 2003 | 748 | 2003 |
A controlled study of adenoviral-vector–mediated gene transfer in the nasal epithelium of patients with cystic fibrosis MR Knowles, KW Hohneker, Z Zhou, JC Olsen, TL Noah, PC Hu, ... New England Journal of Medicine 333 (13), 823-831, 1995 | 719 | 1995 |
Na+ transport in cystic fibrosis respiratory epithelia. Abnormal basal rate and response to adenylate cyclase activation. RC Boucher, MJ Stutts, MR Knowles, L Cantley, JT Gatzy The Journal of clinical investigation 78 (5), 1245-1252, 1986 | 695 | 1986 |
Mutations in DNAH5 cause primary ciliary dyskinesia and randomization of left–right asymmetry H Olbrich, K Häffner, A Kispert, A Völkel, A Volz, G Sasmaz, R Reinhardt, ... Nature genetics 30 (2), 143-144, 2002 | 653 | 2002 |
Abnormal ion permeation through cystic fibrosis respiratory epithelium MR Knowles, MJ Stutts, A Spock, N Fischer, JT Gatzy, RC Boucher Science 221 (4615), 1067-1070, 1983 | 625 | 1983 |
Genetic modifiers of lung disease in cystic fibrosis ML Drumm, MW Konstan, MD Schluchter, A Handler, R Pace, F Zou, ... New England Journal of Medicine 353 (14), 1443-1453, 2005 | 616 | 2005 |
Activation by extracellular nucleotides of chloride secretion in the airway epithelia of patients with cystic fibrosis MR Knowles, LL Clarke, RC Boucher New England Journal of Medicine 325 (8), 533-538, 1991 | 603 | 1991 |
Primary ciliary dyskinesia. Recent advances in diagnostics, genetics, and characterization of clinical disease MR Knowles, LA Daniels, SD Davis, MA Zariwala, MW Leigh American journal of respiratory and critical care medicine 188 (8), 913-922, 2013 | 587 | 2013 |
Clinical and genetic aspects of primary ciliary dyskinesia/Kartagener syndrome MW Leigh, JE Pittman, JL Carson, TW Ferkol, SD Dell, SD Davis, ... Genetics in Medicine 11 (7), 473-487, 2009 | 568 | 2009 |
A novel mutation in the cystic fibrosis gene in patients with pulmonary disease but normal sweat chloride concentrations WE Highsmith, LH Burch, Z Zhou, JC Olsen, TE Boat, A Spock, JD Gorvoy, ... New England Journal of Medicine 331 (15), 974-980, 1994 | 521 | 1994 |
Congenital heart disease and other heterotaxic defects in a large cohort of patients with primary ciliary dyskinesia MP Kennedy, H Omran, MW Leigh, S Dell, L Morgan, PL Molina, ... Circulation 115 (22), 2814-2821, 2007 | 512 | 2007 |
Recommendations for the classification of diseases as CFTR-related disorders C Bombieri, M Claustres, K De Boeck, N Derichs, J Dodge, E Girodon, ... Journal of Cystic Fibrosis 10, S86-S102, 2011 | 489 | 2011 |
Longitudinal analysis of pulmonary function decline in patients with cystic fibrosis M Corey, L Edwards, H Levison, M Knowles The Journal of pediatrics 131 (6), 809-814, 1997 | 444 | 1997 |
A pilot study of aerosolized amiloride for the treatment of lung disease in cystic fibrosis MR Knowles, NL Church, WE Waltner, JR Yankaskas, P Gilligan, M King, ... New England Journal of Medicine 322 (17), 1189-1194, 1990 | 407 | 1990 |