|Aminoglycoside antibiotics restore CFTR function by overcoming premature stop mutations|
M Howard, RA Frizzell, DM Bedwell
Nature medicine 2 (4), 467-469, 1996
|Suppression of a CFTR premature stop mutation in a bronchial epithelial cell line|
DM Bedwell, A Kaenjak, DJ Benos, Z Bebok, JK Bubien, J Hong, ...
Nature medicine 3 (11), 1280-1284, 1997
|Aminoglycoside antibiotics mediate context-dependent suppression of termination codons in a mammalian translation system.|
M Manuvakhova, K Keeling, DM Bedwell
RNA 6 (7), 1044-1055, 2000
|The Efficiency of Translation Termination is Determined by a Synergistic Interplay Between Upstream and Downstream Sequences in Saccharomyces cerevisiae|
B Bonetti, L Fu, J Moon, DM Bedwell
Journal of molecular biology 251 (3), 334-345, 1995
|Evidence that systemic gentamicin suppresses premature stop mutations in patients with cystic fibrosis|
JP Clancy, Z Bebok, F Ruiz, C King, J Jones, L Walker, H Greer, J Hong, ...
American journal of respiratory and critical care medicine 163 (7), 1683-1692, 2001
|PTC124 is an orally bioavailable compound that promotes suppression of the human CFTR-G542X nonsense allele in a CF mouse model|
M Du, X Liu, EM Welch, S Hirawat, SW Peltz, DM Bedwell
Proceedings of the National Academy of Sciences 105 (6), 2064-2069, 2008
|The spc ribosomal protein operon of Eschenchia coli: sequence and cotranscriptlon of the rlbosomal protein genes and a protein export gene|
DP Cerretti, D Dean, GR Davis, DM Bedwell, M Nomura
Nucleic acids research 11 (9), 2599-2616, 1983
|GTP hydrolysis by eRF3 facilitates stop codon decoding during eukaryotic translation termination|
J Salas-Marco, DM Bedwell
Molecular and cellular biology 24 (17), 7769-7778, 2004
|Gentamicin-mediated suppression of Hurler syndrome stop mutations restores a low level of α-l-iduronidase activity and reduces lysosomal glycosaminoglycan …|
KM Keeling, DA Brooks, JJ Hopwood, P Li, JN Thompson, DM Bedwell
Human molecular genetics 10 (3), 291-300, 2001
|Therapeutics Based on Stop Codon Readthrough|
KM Keeling, X Xue, G Gunn, DM Bedwell
Annual Review of Genomics and Human Genetics 15 (1), 2014
|Aminoglycoside suppression of a premature stop mutation in a Cftr–/– mouse carrying a human CFTR-G542X transgene|
M Du, JR Jones, J Lanier, KM Keeling, RJ Lindsey, A Tousson, Z Beb, ...
Journal of molecular medicine 80 (9), 595-604, 2002
|Leaky termination at premature stop codons antagonizes nonsense-mediated mRNA decay in S. cerevisiae|
KIMM KEELING, J LANIER, M DU, JOE SALAS-MARCO, LIN GAO, ...
RNA 10 (4), 691-703, 2004
|The vacuolar Ca2+/H+ exchanger Vcx1p/Hum1p tightly controls cytosolic Ca2+ levels in S. cerevisiae|
A Miseta, R Kellermayer, DP Aiello, L Fu, DM Bedwell
FEBS letters 451 (2), 132-136, 1999
|Clinically relevant aminoglycosides can suppress disease-associated premature stop mutations in the IDUAand P53 cDNAs in a mammalian translation system|
KM Keeling, DM Bedwell
Journal of molecular medicine 80 (6), 367-376, 2002
|Characterization of defects in ion transport and tissue development in cystic fibrosis transmembrane conductance regulator (CFTR)-knockout rats|
KL Tuggle, SE Birket, X Cui, J Hong, J Warren, L Reid, A Chambers, D Ji, ...
PloS one 9 (3), e91253, 2014
|Discrimination between defects in elongation fidelity and termination efficiency provides mechanistic insights into translational readthrough|
J Salas-Marco, DM Bedwell
Journal of molecular biology 348 (4), 801-815, 2005
|Synthetic aminoglycosides efficiently suppress cystic fibrosis transmembrane conductance regulator nonsense mutations and are enhanced by ivacaftor|
X Xue, V Mutyam, L Tang, S Biswas, M Du, LA Jackson, Y Dai, ...
American journal of respiratory cell and molecular biology 50 (4), 805-816, 2014
|Ataluren stimulates ribosomal selection of near-cognate tRNAs to promote nonsense suppression|
B Roy, WJ Friesen, Y Tomizawa, JD Leszyk, J Zhuo, B Johnson, J Dakka, ...
Proceedings of the National Academy of Sciences 113 (44), 12508-12513, 2016
|Nucleotide sequence of the alpha ribosomal protein operon of Escherichia coli|
D Bedwell, G Davis, M Gosink, L Post, M Nomura, H Kestler, JM Zengel, ...
Nucleic acids research 13 (11), 3891-3902, 1985
|Premature translation termination mutations are efficiently suppressed in a highly conserved region of yeast Ste6p, a member of the ATP-binding cassette (ABC) transporter family.|
K Fearon, V McClendon, B Bonetti, DM Bedwell
Journal of Biological Chemistry 269 (27), 17802-17808, 1994