cystic fibrosis airway epithelium. Nature 1988; 331: 358-360. 
27. Li M, McCann JD, Anderson MP , Clancy JP, Liedtke CM, Nairn AC, Greengard 
P, Welsh M J . Regulation of chloride channels by protein kinase C in nor- 
mal and cystic fibrosis airway epithelia. Science 1989; 244: 1353. 
28. Collins FS . Cystic fibrosis: molecular biology and therapeutic implica- 
tions. Science 1992; 256: 774-779. 
29. Welsh M J , Anderson MP, Rich DP, Berger HA, Denning GM, Ostedgaard LS , 
Sheppard DN, Cheng SH, Gregory RJ , Smith AE. Cystic fibrosis transmem- 
brane conductance regulator: A chloride channel with novel regulation. 
Neuron 1992; 8: 821-829. 
30. Rennard SI, Beckmann JD, Robbins RA. Biology of Airway Epithelial Cells. 
In Crystal RG, West JB. The Lung. Scientific Foundations . New York: Ra- 
ven Press; 1991: 157-167. 
31. Deffebach ME, Widdicombe J. The Bronchial Circulation. In: Crystal RG , 
West JB, The Lung. Scientific Foundations. New York: Raven Press; 1991: 
157-167. 
32. Zielenski J, Rozmahel R, Bozon D, Kerem BS , Grzelczak Z, Riordan JR, 
Rommens J, Tsui L-C. Genomic DNA sequence of the cystic fibrosis trans- 
membrane conductance regulator (CFTR) gene. Genomics 1991; 10: 214-228. 
33. Kerem E, Corey M, Kerem BS , Rommens J, Markiewicz D, Levison H, Tsui 
L-C, Durie P. The relation between genotype and phenotype in cystic fi- 
brosis: analysis of the most common mutation (delta F508). N Engl J Med 
1990; 323: 1517-1522. 
34. Cutting GR, Kasch LM, Rosenstein BJ , Tsui L-C, Kazazian HH,Jr., An- 
tonarakis SE. Two patients with cystic fibrosis, nonsense mutations in 
each cystic fibrosis gene, and mild pulmonary disease. N Engl J Med 
1990; 323: 1685-1689. 
35. Hamosh A, Trapnell BC, Zeitlin PL, Montrose -Rafizadeh C, Rosenstein BJ , 
Crystal RG, Cutting GR. Severe deficiency of cystic fibrosis trans- 
membrane conductance regulator messenger RNA carrying nonsense mutations 
R553X and W1316X in respiratory epithelial cells of patients with cystic 
fibrosis. J Clin Invest 1991; 88: 1880-1885. 
36. Hyde SC, Emsley P, Hartshorn MJ , Mimmack MM, Gileadi U, Pearce SR, Gal- 
lagher MP, Gill DR, Hubbard RE, Higgins CF. Structural model of 
ATP-binding proteins associated with cystic fibrosis, multidrug resis- 
tance and bacterial transport. Nature 1990; 346: 362-365. 
37. Cheng SH, Gregory RJ , Marshall J, Paul S, Souza DW, White GA, O' Riordan 
CR, Smith AE. Defective intracellular transport and processing of CFTR 
is the molecular basis of most cystic fibrosis. Cell 1990; 63: 827-834. 
38. Crawford I, Maloney PC, Zeitlin PL, Guggino WB, Hyde SC, Turley H, Gat- 
ter KC, Harris A, Higgins CF. Immunocytochemical localization of the 
Recombinant DNA Research, Volume 20 
[217] 
