Year |
Citation |
Score |
2024 |
Mangkalaphiban K, Fu L, Du M, Thrasher K, Keeling KM, Bedwell DM, Jacobson A. Extended stop codon context predicts nonsense codon readthrough efficiency in human cells. Nature Communications. 15: 2486. PMID 38509072 DOI: 10.1038/s41467-024-46703-z |
0.664 |
|
2023 |
Siddiqui A, Saxena A, Echols J, Havasi V, Fu L, Keeling KM. RNA binding proteins PTBP1 and HNRNPL regulate mRNA decay. Heliyon. 9: e22281. PMID 38045134 DOI: 10.1016/j.heliyon.2023.e22281 |
0.31 |
|
2023 |
Staedtke V, Anstett K, Bedwell D, Giovannini M, Keeling K, Kesterson R, Kim Y, Korf B, Leier A, McManus ML, Sarnoff H, Vitte J, Walker JA, Plotkin SR, Wallis D. Gene-targeted therapy for neurofibromatosis and schwannomatosis: The path to clinical trials. Clinical Trials (London, England). 17407745231207970. PMID 37937606 DOI: 10.1177/17407745231207970 |
0.584 |
|
2023 |
Chen J, Thrasher K, Fu L, Wang W, Aghamohammadzadeh S, Wen H, Tang L, Keeling KM, Falk Libby E, Bedwell DM, Rowe SM. The synthetic aminoglycoside ELX-02 induces readthrough of G550X-CFTR producing super-functional protein that can be further enhanced by CFTR modulators. American Journal of Physiology. Lung Cellular and Molecular Physiology. PMID 37014818 DOI: 10.1152/ajplung.00038.2023 |
0.641 |
|
2023 |
Siddiqui A, Dundar H, Sharma J, Kaczmarczyk A, Echols J, Dai Y, Sun CR, Du M, Liu Z, Zhao R, Wood T, Sanders S, Rasmussen L, Bostwick JR, Augelli-Szafran C, ... ... Keeling KM, et al. Triamterene Functions as an Effective Nonsense Suppression Agent for MPS I-H (Hurler Syndrome). International Journal of Molecular Sciences. 24. PMID 36901952 DOI: 10.3390/ijms24054521 |
0.615 |
|
2022 |
Wang D, Xue X, Gunn G, Du M, Siddiqui A, Weetall M, Keeling KM. Ataluren suppresses a premature termination codon in an MPS I-H mouse. Journal of Molecular Medicine (Berlin, Germany). PMID 35857082 DOI: 10.1007/s00109-022-02232-0 |
0.449 |
|
2021 |
Sharma J, Du M, Wong E, Mutyam V, Li Y, Chen J, Wangen J, Thrasher K, Fu L, Peng N, Tang L, Liu K, Mathew B, Bostwick RJ, Augelli-Szafran CE, ... ... Keeling KM, et al. A small molecule that induces translational readthrough of CFTR nonsense mutations by eRF1 depletion. Nature Communications. 12: 4358. PMID 34272367 DOI: 10.1038/s41467-021-24575-x |
0.68 |
|
2020 |
Echols J, Siddiqui A, Dai Y, Havasi V, Sun R, Kaczmarczyk A, Keeling KM. A regulated NMD mouse model supports NMD inhibition as a viable therapeutic option to treat genetic diseases. Disease Models & Mechanisms. PMID 34005073 DOI: 10.1242/dmm.044891 |
0.33 |
|
2020 |
Echols J, Siddiqui A, Dai Y, Havasi V, Sun R, Kaczmarczyk A, Keeling KM. A regulated NMD mouse model supports NMD inhibition as a viable therapeutic option to treat genetic diseases. Disease Models & Mechanisms. PMID 32737261 DOI: 10.1242/Dmm.044891 |
0.438 |
|
2020 |
Sharma J, Keeling KM, Rowe SM. Pharmacological approaches for targeting cystic fibrosis nonsense mutations. European Journal of Medicinal Chemistry. 200: 112436. PMID 32512483 DOI: 10.1016/J.Ejmech.2020.112436 |
0.487 |
|
2020 |
Leier A, Bedwell DM, Chen AT, Dickson G, Keeling KM, Kesterson RA, Korf BR, Marquez Lago TT, Müller UF, Popplewell L, Zhou J, Wallis D. Mutation-Directed Therapeutics for Neurofibromatosis Type I. Molecular Therapy. Nucleic Acids. 20: 739-753. PMID 32408052 DOI: 10.1016/J.Omtn.2020.04.012 |
0.672 |
|
2020 |
Keeling KM, Bedwell DM. Finding sense in the context. Elife. 9. PMID 32202493 DOI: 10.7554/Elife.55960 |
0.597 |
|
2019 |
Bedwell D, Siddiqui A, Dundar H, Echols J, Du M, Rasmussen L, Robert Bostwick J, Suto M, Keeling K. Triamterene normalizes glycosaminoglycan accumulation in an IDUA-W402X mouse model of MPS I (Hurler syndrome) via nonsense suppression Molecular Genetics and Metabolism. 126: S30. DOI: 10.1016/J.Ymgme.2018.12.054 |
0.597 |
|
2017 |
Xue X, Mutyam V, Thakerar A, Mobley J, Bridges RJ, Rowe SM, Keeling KM, Bedwell DM. Identification of the Amino Acids Inserted During Suppression of CFTR Nonsense Mutations and Determination of Their Functional Consequences. Human Molecular Genetics. PMID 28575328 DOI: 10.1093/Hmg/Ddx196 |
0.7 |
|
2017 |
Bedwell D, Gunn G, Siddiqui A, Du M, Bostwick B, Suto M, Rowe S, Keeling KM. Identification of drugs to treat MPS I caused by nonsense mutations Molecular Genetics and Metabolism. 120: S25. DOI: 10.1016/J.Ymgme.2016.11.035 |
0.673 |
|
2016 |
Keeling KM. Nonsense Suppression as an Approach to Treat Lysosomal Storage Diseases. Diseases (Basel, Switzerland). 4. PMID 28367323 DOI: 10.3390/Diseases4040032 |
0.481 |
|
2016 |
Roy B, Friesen WJ, Tomizawa Y, Leszyk JD, Zhuo J, Johnson B, Dakka J, Trotta CR, Xue X, Mutyam V, Keeling KM, Mobley JA, Rowe SM, Bedwell DM, Welch EM, et al. Ataluren stimulates ribosomal selection of near-cognate tRNAs to promote nonsense suppression. Proceedings of the National Academy of Sciences of the United States of America. PMID 27702906 DOI: 10.1073/Pnas.1605336113 |
0.715 |
|
2016 |
Mutyam V, Du M, Xue X, Keeling KM, White EL, Bostwick JR, Rasmussen L, Liu B, Mazur M, Hong JS, Falk Libby E, Liang F, Shang H, Mense M, Suto MJ, et al. Discovery of Clinically Approved Agents That Promote Suppression of CFTR Nonsense Mutations. American Journal of Respiratory and Critical Care Medicine. PMID 27104944 DOI: 10.1164/Rccm.201601-0154Oc |
0.671 |
|
2015 |
Bedwell DM, Wang D, Welch EM, Keeling KM. The nonsense suppression drug PTC124 restored alpha-l-iduronidase activity and reduces glycosaminoglycan accumulation in MPS IH mice carrying the Idua-W402X mutation Molecular Genetics and Metabolism. 114: S20. DOI: 10.1016/J.Ymgme.2014.12.026 |
0.665 |
|
2014 |
Keeling KM, Xue X, Gunn G, Bedwell DM. Therapeutics based on stop codon readthrough. Annual Review of Genomics and Human Genetics. 15: 371-94. PMID 24773318 DOI: 10.1146/Annurev-Genom-091212-153527 |
0.732 |
|
2014 |
Gunn G, Dai Y, Du M, Belakhov V, Kandasamy J, Schoeb TR, Baasov T, Bedwell DM, Keeling KM. Long-term nonsense suppression therapy moderates MPS I-H disease progression. Molecular Genetics and Metabolism. 111: 374-81. PMID 24411223 DOI: 10.1016/J.Ymgme.2013.12.007 |
0.682 |
|
2014 |
Gunn GG, Dai Y, Du M, Beklakhov V, Kandasamy J, Schoeb TR, Baasov T, Bedwell DM, Keeling KM. Long-term nonsense suppression therapy with NB84 moderates MPS IH disease progression Molecular Genetics and Metabolism. 111: S50. DOI: 10.1016/J.Ymgme.2013.12.106 |
0.592 |
|
2013 |
Keeling KM, Wang D, Dai Y, Murugesan S, Chenna B, Clark J, Belakhov V, Kandasamy J, Velu SE, Baasov T, Bedwell DM. Attenuation of nonsense-mediated mRNA decay enhances in vivo nonsense suppression. Plos One. 8: e60478. PMID 23593225 DOI: 10.1371/Journal.Pone.0060478 |
0.728 |
|
2012 |
Keeling KM, Wang D, Conard SE, Bedwell DM. Suppression of premature termination codons as a therapeutic approach. Critical Reviews in Biochemistry and Molecular Biology. 47: 444-63. PMID 22672057 DOI: 10.3109/10409238.2012.694846 |
0.795 |
|
2012 |
Wang D, Belakhov V, Kandasamy J, Baasov T, Li SC, Li YT, Bedwell DM, Keeling KM. The designer aminoglycoside NB84 significantly reduces glycosaminoglycan accumulation associated with MPS I-H in the Idua-W392X mouse. Molecular Genetics and Metabolism. 105: 116-25. PMID 22056610 DOI: 10.1016/J.Ymgme.2011.10.005 |
0.709 |
|
2011 |
Keeling KM, Bedwell DM. Suppression of nonsense mutations as a therapeutic approach to treat genetic diseases. Wiley Interdisciplinary Reviews. Rna. 2: 837-52. PMID 21976286 DOI: 10.1002/Wrna.95 |
0.707 |
|
2011 |
Lazrak A, Jurkuvenaite A, Chen L, Keeling KM, Collawn JF, Bedwell DM, Matalon S. Enhancement of alveolar epithelial sodium channel activity with decreased cystic fibrosis transmembrane conductance regulator expression in mouse lung. American Journal of Physiology. Lung Cellular and Molecular Physiology. 301: L557-67. PMID 21743028 DOI: 10.1152/Ajplung.00094.2011 |
0.585 |
|
2010 |
Wang D, Shukla C, Liu X, Schoeb TR, Clarke LA, Bedwell DM, Keeling KM. Characterization of an MPS I-H knock-in mouse that carries a nonsense mutation analogous to the human IDUA-W402X mutation. Molecular Genetics and Metabolism. 99: 62-71. PMID 19751987 DOI: 10.1016/J.Ymgme.2009.08.002 |
0.69 |
|
2010 |
Wang D, Shukla C, Liu X, Schoeb TR, Clarke LA, Bedwell DM, Keeling KM. Corrigendum to "Characterization of an MPS I-H knock-in mouse that carries a nonsense mutation analogous to the human IDUA-W402X mutation" [Mol. Genet. Metab. 99 (2010) 62-71] (DOI:10.1016/j.ymgme.2009.08.002) Molecular Genetics and Metabolism. 99: 439. DOI: 10.1016/J.Ymgme.2009.12.013 |
0.702 |
|
2009 |
Du M, Keeling KM, Fan L, Liu X, Bedwell DM. Poly-L-aspartic acid enhances and prolongs gentamicin-mediated suppression of the CFTR-G542X mutation in a cystic fibrosis mouse model. The Journal of Biological Chemistry. 284: 6885-92. PMID 19136563 DOI: 10.1074/Jbc.M806728200 |
0.714 |
|
2009 |
Bedwell D, Wang D, Shukla C, Liu X, Yeh S, Welch E, Schoeb T, Keeling K. 12. Suppression of a nonsense mutation in a mouse model of Hurler syndrome Molecular Genetics and Metabolism. 96: S14. DOI: 10.1016/J.Ymgme.2008.11.013 |
0.692 |
|
2008 |
Fan-Minogue H, Du M, Pisarev AV, Kallmeyer AK, Salas-Marco J, Keeling KM, Thompson SR, Pestova TV, Bedwell DM. Distinct eRF3 requirements suggest alternate eRF1 conformations mediate peptide release during eukaryotic translation termination. Molecular Cell. 30: 599-609. PMID 18538658 DOI: 10.1016/J.Molcel.2008.03.020 |
0.682 |
|
2006 |
Kallmeyer AK, Keeling KM, Bedwell DM. Eukaryotic release factor 1 phosphorylation by CK2 protein kinase is dynamic but has little effect on the efficiency of translation termination in Saccharomyces cerevisiae. Eukaryotic Cell. 5: 1378-87. PMID 16896221 DOI: 10.1128/Ec.00073-06 |
0.723 |
|
2006 |
Keeling KM, Salas-Marco J, Osherovich LZ, Bedwell DM. Tpa1p is part of an mRNP complex that influences translation termination, mRNA deadenylation, and mRNA turnover in Saccharomyces cerevisiae. Molecular and Cellular Biology. 26: 5237-48. PMID 16809762 DOI: 10.1128/Mcb.02448-05 |
0.771 |
|
2006 |
Du M, Keeling KM, Fan L, Liu X, Kovaçs T, Sorscher E, Bedwell DM. Clinical doses of amikacin provide more effective suppression of the human CFTR-G542X stop mutation than gentamicin in a transgenic CF mouse model. Journal of Molecular Medicine (Berlin, Germany). 84: 573-82. PMID 16541275 DOI: 10.1007/S00109-006-0045-5 |
0.69 |
|
2006 |
Kellermayer R, Szigeti R, Keeling KM, Bedekovics T, Bedwell DM. Aminoglycosides as potential pharmacogenetic agents in the treatment of Hailey-Hailey disease. The Journal of Investigative Dermatology. 126: 229-31. PMID 16417242 DOI: 10.1038/Sj.Jid.5700031 |
0.578 |
|
2005 |
Keeling KM, Bedwell DM. Pharmacological suppression of premature stop mutations that cause genetic diseases Current Pharmacogenomics. 3: 259-269. DOI: 10.2174/157016005774913149 |
0.719 |
|
2004 |
Keeling KM, Lanier J, Du M, Salas-Marco J, Gao L, Kaenjak-Angeletti A, Bedwell DM. Leaky termination at premature stop codons antagonizes nonsense-mediated mRNA decay in S. cerevisiae. Rna (New York, N.Y.). 10: 691-703. PMID 15037778 DOI: 10.1261/Rna.5147804 |
0.789 |
|
2002 |
Du M, Jones JR, Lanier J, Keeling KM, Lindsey JR, Tousson A, Bebök Z, Whitsett JA, Dey CR, Colledge WH, Evans MJ, Sorscher EJ, Bedwell DM. Aminoglycoside suppression of a premature stop mutation in a Cftr-/- mouse carrying a human CFTR-G542X transgene. Journal of Molecular Medicine (Berlin, Germany). 80: 595-604. PMID 12226741 DOI: 10.1007/S00109-002-0363-1 |
0.715 |
|
2002 |
Keeling KM, Bedwell DM. Clinically relevant aminoglycosides can suppress disease-associated premature stop mutations in the IDUA and P53 cDNAs in a mammalian translation system. Journal of Molecular Medicine (Berlin, Germany). 80: 367-76. PMID 12072912 DOI: 10.1007/S00109-001-0317-Z |
0.737 |
|
2001 |
Keeling KM, Brooks DA, Hopwood JJ, Li P, Thompson JN, Bedwell DM. Gentamicin-mediated suppression of Hurler syndrome stop mutations restores a low level of alpha-L-iduronidase activity and reduces lysosomal glycosaminoglycan accumulation. Human Molecular Genetics. 10: 291-9. PMID 11159948 DOI: 10.1093/Hmg/10.3.291 |
0.672 |
|
2000 |
Manuvakhova M, Keeling K, Bedwell DM. Aminoglycoside antibiotics mediate context-dependent suppression of termination codons in a mammalian translation system Rna. 6: 1044-1055. PMID 10917599 DOI: 10.1017/S1355838200000716 |
0.688 |
|
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