Michael R. Knowles, M.D. 
6. Knowles, M.R., N.L. Church, W.E. Waltner, J.R. Yankaskas, P. Gilligan, M. King, L.J. Edwards 
R.W. Helms, and R.C. Boucher. A pilot study of aerosolized amiloride for the treatment of lung 
disease in cystic fibrosis. N. Engl. J. Med. 322:1189-1194, 1990. 
7. Kobayashi, K., M.R. Knowles, R.C. Boucher, W.E. O’Brien, and A.L. Beaudet. Benign missense 
mutations in the cystic fibrosis gene. Am. J. Hum. Genet. 47:611-615, 1990. 
8. Highsmith, Jr., W.E., G.L Chong, H.T. Orr, T.R. Perry, D. Schaid, R. Farber, K Wagner, M.R. 
Knowles, W.J. Warwick, L.M. Silverman, and S.N. Thibodeau. Frequency of the APhe 508 mutation 
and correlation with XV.2c/KM-19 haplotypes in an American population of cystic fibrosis patients: 
results of a collaborative study. Clin. Chem. 36:1741-1746, 1990. 
9. Carson, J.L., A.M. Collier, T.M. Gambling, M.R. Knowles, and R.C. Boucher. Ultrastructure of 
airway epithelial cell membranes among patients with cystic fibrosis. Human Path. 21:640-647, 1990. 
10. Boundy, M.G., D. Leith, and M. Knowles. Dispersion and size distribution of amiloride by 
metered dose and dry powder inhalers./. Aerosol Med. 3:233-241, 1990. 
11. Knowles, M.R., L.L, Clarke, and R.C. Boucher. Activation by extracullular nucleotides of chloride 
secretion in the airway epithelia of patients with cystic fibrosis. N. Engl. J. Med. 325:533-538, 1991. 
12. Strong, T.V., L.S. Smit, S.V. Turpin, J.L. Cole, C.T. Hon, T.L. Petty, M.W. Craig, E.C. Rosenaw, 
III, L.-C. Tsui, M.C. Iannuzzi, M.R. Knowles, and F.S. Collins. Cystic fibrosis gene in two sisters with 
mild disease and normal sweat electrolyte levels. N. Engl. J. Med. 325: 1630-1634, 1991. 
13. Gowen, C.W., Jr., M.A. Gowen, and M.R. Knowles. Colonic transepithelial potential difference in 
infants with cystic fibrosis./. Pediatr. 118: 412-415, 1991. 
14. Kilby, J.M., P.H. Gilligan, J.R. Yankaskas, W.E. Highsmith, Jr., L.J. Edwards, and M.R. Knowles. 
Nontuberculous mycobacteria in adult patients with cystic fibrosis. Chest 102:70-75, 1992. 
15. Stutts, M.J., M.R. Knowles, T. Chinet, and R.C. Boucher. Abnormal ion transport in cystic fibrosis 
airway epithelium. In: The Airway Epithelium. Physiology. Pathophysiology, and Pharmacology . S.G. 
Farmer and D. W. P. Hay, editors. Marcel Dekker, Inc., New York. 301-334, 1991. 
16. Knowles, M.R., L.L. Clarke, and R.C. Boucher. Extracellular ATP and UTP induce chloride 
secretion in nasal epithelia of CF patients and normal subjects in vivo. Chest 101:60S-63S, 1992. 
17. Knowles, M.R., N.L. Church, W.E. Waltner, D. Jones, J.T. Gatzy, and R.C. Boucher. Amiloride in 
cystic fibrosis: safety, pharmacokinetics, and efficacy in the treatment of pulmonary disease. In: 
Amiloride and its Analogs: Unique Cation Transport Inhibitors . VCH Publishers, New York (In 
Press) 1992. 
18. Turpin, S.V., and M.R. Knowles. Treatment of Pulmonary disease in patients with cystic fibrosis. 
In: Lung Biology in Health and Disease: Cystic Fibrosis . P.B. Davis, editor, Marcel Dekker Press Inc. 
(In Press) 1992. 
19. Tomkiewicz, R. P., E. M. App, R. C. Boucher, M. R. Knowles, and M. King. Amiloride inhalation 
therapy and its influence on sputum electrolyte composition in cystic fibrosis. (Submitted) Am. Rev. 
Respir. Dis. 1992. 
20. Whittier, S., R. L. Hopfer, M. R. Knowles, and P. H. Gilligan. Improved recovery of mycobacteria 
from respiratory secretions of patients with cystic fibrosis. (Submitted) American Society for 
Microbiology, 1992. 
21. Highsmith, W.E., L.H. Burch, T.H. Boat, A. Spock, J. Gorvay, L. Quitted, K.J. Freidman, L.M. 
Silverman, R.C. Boucher, and M.R. Knowles. A novel cystic fibrosis gene mutation that is common in 
patients with normal sweat chloride concentrations. (Submitted) N. Engl. J. Med. 1992 
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Recombinant DNA Research, Volume 17 
