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 | 426 | 2000 |
Therapeutics based on stop codon readthrough KM Keeling, X Xue, G Gunn, DM Bedwell Annual review of genomics and human genetics 15, 371-394, 2014 | 340 | 2014 |
Preliminary crystallographic studies of four crystal forms of serum albumin DC Carter, B Chang, JX Ho, K Keeling, Z Krishnasami European Journal of Biochemistry 226 (3), 1049-1052, 1994 | 283 | 1994 |
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 | 220 | 2001 |
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 | 219 | 2016 |
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 | 219 | 2002 |
Three‐Dimensional structure of schistosoma japonicum glutathione s‐transferase fused with a six‐amino acid conserved neutralizing epitope of gp41 from hiv K Lim, JX Ho, K Keeling, GL Gilliland, X Ji, F Rüker, DC Carter Protein Science 3 (12), 2233-2244, 1994 | 208 | 1994 |
Leaky termination at premature stop codons antagonizes nonsense-mediated mRNA decay in S. cerevisiae KM Keeling, J Lanier, M Du, JOE Salas-Marco, LIN Gao, ... Rna 10 (4), 691-703, 2004 | 192 | 2004 |
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 | 158 | 2002 |
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 | 123 | 2012 |
Suppression of nonsense mutations as a therapeutic approach to treat genetic diseases KM Keeling, DM Bedwell Wiley Interdisciplinary Reviews: RNA 2 (6), 837-852, 2011 | 119 | 2011 |
Attenuation of nonsense-mediated mRNA decay enhances in vivo nonsense suppression KM Keeling, D Wang, Y Dai, S Murugesan, B Chenna, J Clark, ... PloS one 8 (4), e60478, 2013 | 109 | 2013 |
Discovery of clinically approved agents that promote suppression of cystic fibrosis transmembrane conductance regulator nonsense mutations V Mutyam, M Du, X Xue, KM Keeling, EL White, JR Bostwick, ... American journal of respiratory and critical care medicine 194 (9), 1092-1103, 2016 | 96 | 2016 |
The designer aminoglycoside NB84 significantly reduces glycosaminoglycan accumulation associated with MPS IH in the Idua-W392X mouse D Wang, V Belakhov, J Kandasamy, T Baasov, SC Li, YT Li, DM Bedwell, ... Molecular genetics and metabolism 105 (1), 116-125, 2012 | 93 | 2012 |
Identification of the amino acids inserted during suppression of CFTR nonsense mutations and determination of their functional consequences X Xue, V Mutyam, A Thakerar, J Mobley, RJ Bridges, SM Rowe, ... Human molecular genetics 26 (16), 3116-3129, 2017 | 87 | 2017 |
Distinct eRF3 requirements suggest alternate eRF1 conformations mediate peptide release during eukaryotic translation termination H Fan-Minogue, M Du, AV Pisarev, AK Kallmeyer, J Salas-Marco, ... Molecular cell 30 (5), 599-609, 2008 | 87 | 2008 |
Lower dimer impurity incorporation may result in higher perfection of HEWL crystals grown in microgravity: A case study DC Carter, K Lim, JX Ho, BS Wright, PD Twigg, TY Miller, J Chapman, ... Journal of crystal growth 196 (2-4), 623-637, 1999 | 87 | 1999 |
Clinical doses of amikacin provide more effective suppression of the human CFTR-G542X stop mutation than gentamicin in a transgenic CF mouse model M Du, KM Keeling, L Fan, X Liu, T Kovaçs, E Sorscher, DM Bedwell Journal of molecular medicine 84, 573-582, 2006 | 86 | 2006 |
Characterization of an MPS IH knock-in mouse that carries a nonsense mutation analogous to the human IDUA-W402X mutation D Wang, C Shukla, X Liu, TR Schoeb, LA Clarke, DM Bedwell, ... Molecular genetics and metabolism 99 (1), 62-71, 2010 | 74 | 2010 |
A small molecule that induces translational readthrough of CFTR nonsense mutations by eRF1 depletion J Sharma, M Du, E Wong, V Mutyam, Y Li, J Chen, J Wangen, K Thrasher, ... Nature communications 12 (1), 4358, 2021 | 73 | 2021 |