M.J. Welsh and A.E. Smith, RAC Application 
Ad2-ORF6/PGK-CFTR 
Item 8 - Professional Experience 
of Key Personnel 
FF Principal Investigator/Program Director (Last, first, middle): M.J. Welsh and A.E. Smith 
Cheng SH, Rich DP, Marshall J, Gregory RJ, Welsh MJ, and Smith AE. Phosphorylation of the R-Domain by 
cAMP-Dependent Protein Kinase Regulates the CFTR Chloride Channel. Cell, 66:1027-1036. 1991. 
Berger HA, Anderson MP, Gregory RJ, Thompson S, Howard PW, Maurer RA, Mulligan R, Smith AE, and 
Welsh MJ. Identification and Regulation of the CFTR-Generated Chloride Channel. J. Clin. Invest. 
M:H22-1431, 1991. 
Anderson MP, Berger HA, Rich DP, Gregory RJ, Smith AE, and Welsh MJ. Nucleoside Triphosphates Are 
Required to Open the CFTR Chloride Channel. Cell £2:775-784, 1991. 
Denning GM, Ostedgaard LS, Cheng SH, Smith AE, and Welsh MJ. Localization of Cystic Fibrosis 
Transmembrane Conductance Regulator in Chloride Secretory Epithelia. J. Clin. Invest., £2:339-349, 
1992. 
DeLisle S and Welsh MJ. Inositol Triphosphate is Required for the Propagation of Calcium, J. Biol. Chem., 
262:7963-7966, 1992. 
Smith JJ, and Welsh MJ. cAMP Stimulates Bicarbonate Secretion Across Normal, But Not Cystic Fibrosis 
Airway Epithelia. J. Clin. Invest.. 89:1148-1153. 1992. 
DeLisle S, Pittet D, Potter DV, Lew PD, and Welsh MJ. InsP 3 and Ins(l,3,4,5)P 4 Act in Synergy to Stimulate 
Influx of Extracellular Ca 2+ in Xenopus Oocytes. Am. J. Physiol. (Cell Physiol.) 21:C1456-C1463, 1992. 
Tilly BC, Winter M, Ostedgaard LS, O'Riordan C, Smith AE, and Welsh MJ, cAMP Dependent Protein 
Kinase Activation of CFTR Chloride Channels in Planar Lipid Bilayers. J. Biol. Chem. 267 :9470-9473. 
1992. 
Welsh MJ, Anderson MP, Rich DP, Berger HA, Denning GM, Ostedgaard LS, Sheppard, DN, Cheng S, 
Gregory RJ, and Smith AE. Cystic Fibrosis Transmembrane Conductance Regulator: A Chloride Channel 
with Novel Regulation. Neuron, £:821-829, 1992. 
Denning GM, Ostedgaard LS, and Welsh MJ. Abnormal Localization of Cystic Fibrosis Transmembrane 
Conductance Regulator in Primary Cultures of Cystic Fibrosis Airway Epithelia. J. Cell Biol., JJL£:55 1- 
559, 1992. 
Sheppard DN and Welsh MJ. Effects of ATP-Sensitive K + Channel Regulators on CFTR Chloride Currents. J. 
Gen. Physiol., 1QQ:573-591, 1992. 
Denning GM, Anderson MP, Amara J, Marshall J, Smith AE, and Welsh MJ. Processing of Mutant CFTR 
(AF508) is Temperature-Sensitive. Nature, 358 :761-764. 1992. 
Sheppard DN and Welsh MJ. Effect of ATP-Sensitive K + Channel Regulators on CFTR Chloride Currents. J. 
Gen. Physiol., JLQQ:573-59 1 , 1992. 
Parys JB, Sernett SW, DeLisle S, Snyder PM, Welsh MJ, and Campbell KP. Isolation, Characterization and 
Localization of the Inositol 1,4,5-Triphosphate Receptor Protein in Xenopus Laevis Oocytes. J. Biol. 
Chem., 262:18776-18782, 1992. 
Anderson MP and Welsh MJ. Regulation by ATP and ADP of CFTR CL Channels Containing Nucleotide- 
Binding Domains. Science, 257 :1701-1704. 1992. 
Ostedgaard LS and Welsh MJ. Partial Purification of the Cystic Fibrosis Transmembrane Conductance 
Regulator. J. Biol. Chem., 267 :26142-26149. 1992. 
Berger HA, Travis SM, and Welsh MJ. Regulation of the CFTR CL Channel by Specific Protein Kinases and 
Protein Phosphatases. J. Biol. Chem., 2£&:2037-2074, 1993. 
Sheppard DN, Rich DP, Ostedgaard LS, Gregory RJ, Smith AE, and Welsh MJ. Mutations in CFTR 
Associated with Mild Disease Form CL Channels with Altered Pore Properties. Nature 362 :160-164. 
1993. 
Teem TL, Berger HA, Ostedgaard LS, Rich DP, Tsui L-C, and Welsh MJ. Identification of Revertants for the 
Cystic Fibrosis AF508 Mutation Using STE6/CFTR Chimeras in Yeast. Cell, 22:335-346, 1993. 
Carson MR and Welsh MJ. 5' Adenylylimidodphosphate (AMP-PNP) Does Not Activate CFTR Chloride 
Channels in Cell-Free Patches of Membrane. Am. J. Physiol. (Lung Cellular and Molecular Physiol.), 
2££:L27-32, 1993. 
Delaney SJ, Rich DP, Thomson SA, Hargrave MR, Welsh MJ, and Wainwright BJ. CFTR Splice Variants 
Are Not Conserved and Fail to Produce CL Channels. Nature Genetics, 4:426-431, 1993. 
Zabner J, Petersen DM, Puga AP, Graham SM, and Welsh MJ. Safety and Efficacy of Repetitive 
Adenvorius-Mediated Transfer of CFTR cDNA to Airway Epithelia of Primates and Cotton Rats. Nature 
Genetics, (in press). 
Zabner J, Couture, LA, Gregory RJ, Graham SM, Smith AE, and Welsh MJ. Adenovirus-Mediated Gene 
Transfer Transiently Corrects the Chloride Transport Defect in Nasal Epithelia of Patients with Cystic 
Fibrosis. Cell, (in press). 
PHS 398 (Rev. 9/91) (Form Page 6) Page 
Number pages consecutively at the bottom throughout the application. Do nofuse suffices such as 3a, 3b. 
Recombinant DNA Research, Volume 18 
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