Aminoglycoside antibiotics restore CFTR function by overcoming premature stop mutations M Howard, RA Frizzell, DM Bedwell Nature medicine 2 (4), 467-469, 1996 | 604 | 1996 |
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 | 429 | 2000 |
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 | 411 | 1997 |
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 | 390 | 1995 |
Therapeutics Based on Stop Codon Readthrough KM Keeling, X Xue, G Gunn, DM Bedwell Annual Review of Genomics and Human Genetics 15 (1), 2014 | 352 | 2014 |
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 | 318 | 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 | 314 | 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 | 275 | 1983 |
GTP hydrolysis by eRF3 facilitates stop codon decoding during eukaryotic translation termination J Salas-Marco, DM Bedwell Molecular and cellular biology, 2004 | 254 | 2004 |
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 | 227 | 2016 |
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 | 222 | 2001 |
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 Bebök, ... Journal of molecular medicine 80, 595-604, 2002 | 217 | 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 | 198 | 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 | 196 | 1999 |
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 | 170 | 2014 |
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 | 169 | 2014 |
Clinically relevant aminoglycosides can suppress disease-associated premature stop mutations in the IDUA and P53 cDNAs in a mammalian translation … KM Keeling, DM Bedwell Journal of molecular medicine 80, 367-376, 2002 | 159 | 2002 |
Expression and function of the K+ channel KCNQ genes in human arteries FL Ng, AJ Davis, TA Jepps, MI Harhun, SY Yeung, A Wan, M Reddy, ... British journal of pharmacology 162 (1), 42-53, 2011 | 152 | 2011 |
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 | 139 | 2005 |
Suppression of premature termination codons as a therapeutic approach KM Keeling, D Wang, SE Conard, DM Bedwell Critical reviews in biochemistry and molecular biology 47 (5), 444-463, 2012 | 126 | 2012 |