Search Results - "Smith, Alan E."

Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive by Denning, Gerene M, Anderson, Matthew P, Amara, Jane F, Marshall, John, Smith, Alan E, Welsh, Michael J

“…Cystic fibrosis transmembrane conductance regulator (CFTR) is a plasma membrane Cl- channel regulated by cyclic AMP-dependent phosphorylation and by…”
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Generation of cAMP-Activated Chloride Currents by Expression of CFTR by Anderson, Matthew P., Rich, Devra P., Gregory, Richard J., Smith, Alan E., Welsh, Michael J.

“…Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) cause cystic fibrosis. In order to evaluate its function, CFTR was expressed in…”
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Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells by Rich, Devra P, Anderson, Matthew P, Gregory, Richard J, Cheng, Seng H, Paul, Sucharita, Jefferson, Douglas M, McCann, John D, Klinger, Katherine W, Smith, Alan E, Welsh, Michael J

“…The cystic fibrosis transmembrane conductance regulator (CFTR) was expressed in cultured cystic fibrosis airway epithelial cells and Cl- channel activation…”
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Expression and characterization of the cystic fibrosis transmembrane conductance regulator by Gregory, Richard J, Cheng, Seng H, Rich, Devra P, Marshall, John, Paul, Sucharita, Hehir, Kathleen, Ostedgaard, Lynda, Klinger, Katherine W, Welsh, Michael J, Smith, Alan E

“…Cystic fibrosis (CF) is a common lethal genetic disease that manifests itself in airway and other epithelial cells as defective chloride ion absorption and…”
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Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis by Welsh, M J, Smith, A E

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Viral Vectors in Gene Therapy by Smith, A E

“…The use of DNA as a drug is both appealing and simple in concept. Indeed in many instances the feasibility of such an approach has been established using model…”
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Production of Human Tissue Plasminogen Activator in Transgenic Mouse Milk by Gordon, Katherine, Lee, Eric, Vitale, James A, Smith, Alan E, Westphal, Heiner, Hennighausen, Lothar

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Mutations in CFTR associated with mild-disease-form CI - channels with altered pore properties by Sheppard, David N, Rich, Devra P, Ostedgaard, Lynda S, Gregory, Richard J, Smith, Alan E, Welsh, Michael J

“…The cystic fibrosis transmembrane conductance regulator (CFTR) is a phosphorylation-regulated C1- channel located in the apical membrane of epithelia. The fact…”
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Detailed analysis of structures and formulations of cationic lipids for efficient gene transfer to the lung by Lee, E R, Marshall, J, Siegel, C S, Jiang, C, Yew, N S, Nichols, M R, Nietupski, J B, Ziegler, R J, Lane, M B, Wang, K X, Wan, N C, Scheule, R K, Harris, D J, Smith, A E, Cheng, S H

“…Cationic lipid-mediated gene transfer of cystic fibrosis transmembrane conductance regulator (CFTR) cDNA represents a promising approach for treatment of…”
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Telling Tales by Smith, Alan E.

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Demonstration that CFTR is a Chloride Channel by Alteration of its Anion Selectivity by Anderson, Matthew P., Gregory, Richard J., Thompson, Simon, Souza, David W., Paul, Sucharita, Mulligan, Richard C., Smith, Alan E., Welsh, Michael J.

“…Expression of the cystic fibrosis transmembrane conductance regulator (CFTR) generates adenosine 3′,5′-monophosphate (cAMP)-regulated chloride channels,…”
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Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis by Cheng, S H, Gregory, R J, Marshall, J, Paul, S, Souza, D W, White, G A, O'Riordan, C R, Smith, A E

“…The gene associated with cystic fibrosis (CF) encodes a membrane-associated, N-linked glycoprotein called CFTR. Mutations were introduced into CFTR at residues…”
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Aerosol and lobar administration of a recombinant adenovirus to individuals with cystic fibrosis. I. Methods, safety, and clinical implications by Joseph, P M, O'Sullivan, B P, Lapey, A, Dorkin, H, Oren, J, Balfour, R, Perricone, M A, Rosenberg, M, Wadsworth, S C, Smith, A E, St George, J A, Meeker, D P

“…Cystic fibrosis (CF), an autosomal recessive disorder resulting from mutations in the cystic fibrosis trans-membrane conductance regulator (CFTR) gene, is the…”
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Phosphorylation of the R domain by cAMP-dependent protein kinase regulates the CFTR chloride channel by Cheng, S H, Rich, D P, Marshall, J, Gregory, R J, Welsh, M J, Smith, A E

“…CFTR, the protein associated with cystic fibrosis, is phosphorylated on serine residues in response to cAMP agonists. Serines 660, 737, 795, and 813 were…”
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Gene therapy—where are we? by Smith, Alan E

“…Smith discusses the history of gene therapy. Although gene therapy is potentially a powerful clinical approach, it still lacks an unequivocal clinical success…”
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Aerosol and lobar administration of a recombinant adenovirus to individuals with cystic fibrosis. II. Transfection efficiency in airway epithelium by Perricone, M A, Morris, J E, Pavelka, K, Plog, M S, O'Sullivan, B P, Joseph, P M, Dorkin, H, Lapey, A, Balfour, R, Meeker, D P, Smith, A E, Wadsworth, S C, St George, J A

“…A phase I clinical trial was conducted in which recombinant adenovirus containing the cystic fibrosis trans-membrane regulator (CFTR) (Ad2/CFTR) was…”
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Safety and efficacy of repetitive adenovirus-mediated transfer of CFTR cDNA to airway epithelia of primates and cotton rats by Zabner, Joseph, Petersen, Deanna M, Puga, Aurita P, Graham, Scott M, Couture, Larry A, Keyes, Lynn D, Lukason, Mike J, St. George, Judith A, Gregory, Richard J, Smith, Alan E, Welsh, Michael J

“…Gene therapy for cystic fibrosis (CF) will require the safe transfer of CFTR cDNA to airway epithelia in vivo. We showed previously that a recombinant…”
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La trama de Víctor: aspectos del arte galdosiano en Miau by Smith, Alan E.

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Effect of Deleting the R Domain on CFTR-Generated Chloride Channels by Rich, Devra P., Gregory, Richard J., Anderson, Matthew P., Manavalan, Parthasarathy, Smith, Alan E., Welsh, Michael J.

“…The cystic fibrosis transmembrane conductance regulator (CFTR), which forms adenosine 3′,5′-monophosphate (cAMP)-regulated chloride channels, is defective…”
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Characterization of an adenovirus gene transfer vector containing an E4 deletion by Armentano, D, Sookdeo, C C, Hehir, K M, Gregory, R J, St George, J A, Prince, G A, Wadsworth, S C, Smith, A E

“…We describe the construction and characterization of an adenovirus type 2 vector, Ad2E4ORF6, which has been modified in the E4 region to contain only open…”
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