Therapeutics Based on Stop Codon Readthrough von Keeling, Kim M, Xue, Xiaojiao, Gunn, Gwen, Bedwell, David M

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Ataluren stimulates ribosomal selection of near-cognate tRNAs to promote nonsense suppression von Roy, Bijoyita, Friesen, Westley J., Tomizawa, Yuki, Leszyk, John D., Zhuo, Jin, Johnson, Briana, Dakka, Jumana, Trotta, Christopher R., Xue, Xiaojiao, Mutyam, Venkateshwar, Keeling, Kim M., Mobley, James A., Rowe, Steven M., Bedwell, David M., Welch, Ellen M., Jacobson, Allan

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Extended stop codon context predicts nonsense codon readthrough efficiency in human cells von Mangkalaphiban, Kotchaphorn, Fu, Lianwu, Du, Ming, Thrasher, Kari, Keeling, Kim M., Bedwell, David M., Jacobson, Allan

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Attenuation of nonsense-mediated mRNA decay enhances in vivo nonsense suppression von Keeling, Kim M, Wang, Dan, Dai, Yanying, Murugesan, Srinivasan, Chenna, Balachandra, Clark, Jeremy, Belakhov, Valery, Kandasamy, Jeyakumar, Velu, Sadanandan E, Baasov, Timor, Bedwell, David M

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A small molecule that induces translational readthrough of CFTR nonsense mutations by eRF1 depletion von Sharma, Jyoti, Du, Ming, Wong, Eric, Mutyam, Venkateshwar, Li, Yao, Chen, Jianguo, Wangen, Jamie, Thrasher, Kari, Fu, Lianwu, Peng, Ning, Tang, Liping, Liu, Kaimao, Mathew, Bini, Bostwick, Robert J., Augelli-Szafran, Corinne E., Bihler, Hermann, Liang, Feng, Mahiou, Jerome, Saltz, Josef, Rab, Andras, Hong, Jeong, Sorscher, Eric J., Mendenhall, Eric M., Coppola, Candice J., Keeling, Kim M., Green, Rachel, Mense, Martin, Suto, Mark J., Rowe, Steven M., Bedwell, David M.

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Suppression of nonsense mutations as a therapeutic approach to treat genetic diseases von Keeling, Kim M., Bedwell, David M.

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Triamterene Functions as an Effective Nonsense Suppression Agent for MPS I-H (Hurler Syndrome) von Siddiqui, Amna, Dundar, Halil, Sharma, Jyoti, Kaczmarczyk, Aneta, Echols, Josh, Dai, Yanying, Sun, Chuanxi Richard, Du, Ming, Liu, Zhong, Zhao, Rui, Wood, Tim, Sanders, Shalisa, Rasmussen, Lynn, Bostwick, James Robert, Augelli-Szafran, Corinne, Suto, Mark, Rowe, Steven M, Bedwell, David M, Keeling, Kim M

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RNA binding proteins PTBP1 and HNRNPL regulate CFTR mRNA decay von Siddiqui, Amna, Saxena, Arpit, Echols, Joshua, Havasi, Viktoria, Fu, Lianwu, Keeling, Kim M.

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A regulated NMD mouse model supports NMD inhibition as a viable therapeutic option to treat genetic diseases von Echols, Josh, Siddiqui, Amna, Dai, Yanying, Havasi, Viktoria, Sun, Richard, Kaczmarczyk, Aneta, Keeling, Kim M.

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Mutation-Directed Therapeutics for Neurofibromatosis Type I von Leier, Andre, Bedwell, David M., Chen, Ann T., Dickson, George, Keeling, Kim M., Kesterson, Robert A., Korf, Bruce R., Marquez Lago, Tatiana T., Müller, Ulrich F., Popplewell, Linda, Zhou, Jiangbing, Wallis, Deeann

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Pharmacological approaches for targeting cystic fibrosis nonsense mutations von Sharma, Jyoti, Keeling, Kim M., Rowe, Steven M.

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Poly-l-aspartic Acid Enhances and Prolongs Gentamicin-mediated Suppression of the CFTR-G542X Mutation in a Cystic Fibrosis Mouse Model von Du, Ming, Keeling, Kim M., Fan, Liming, Liu, Xiaoli, Bedwell, David M.

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Nonsense Suppression as an Approach to Treat Lysosomal Storage Diseases von Keeling, Kim M

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Identification of the amino acids inserted during suppression of CFTR nonsense mutations and determination of their functional consequences von Xue, Xiaojiao, Mutyam, Venkateshwar, Thakerar, Amita, Mobley, James, Bridges, Robert J, Rowe, Steven M, Keeling, Kim M, Bedwell, David M

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Ataluren suppresses a premature termination codon in an MPS I-H mouse von Wang, Dan, Xue, Xiaojiao, Gunn, Gwen, Du, Ming, Siddiqui, Amna, Weetall, Marla, Keeling, Kim M.

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The synthetic aminoglycoside ELX-02 induces readthrough of G550X-CFTR producing superfunctional protein that can be further enhanced by CFTR modulators von Chen, Jianguo, Thrasher, Kari, Fu, Lianwu, Wang, Wei, Aghamohammadzadeh, Soheil, Wen, Hui, Tang, Liping, Keeling, Kim M, Falk Libby, Emily, Bedwell, David M, Rowe, Steven M

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Distinct eRF3 Requirements Suggest Alternate eRF1 Conformations Mediate Peptide Release during Eukaryotic Translation Termination von Fan-Minogue, Hua, Du, Ming, Pisarev, Andrey V., Kallmeyer, Adam K., Salas-Marco, Joe, Keeling, Kim M., Thompson, Sunnie R., Pestova, Tatyana V., Bedwell, David M.

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Clinical doses of amikacin provide more effective suppression of the human CFTR-G542X stop mutation than gentamicin in a transgenic CF mouse model von Du, Ming, Keeling, Kim M, Fan, Liming, Liu, Xiaoli, Kovaçs, Timea, Sorscher, Eric, Bedwell, David M

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Tpa1p Is Part of an mRNP Complex That Influences Translation Termination, mRNA Deadenylation, and mRNA Turnover in Saccharomyces cerevisiae von Keeling, Kim M., Salas-Marco, Joe, Osherovich, Lev Z., Bedwell, David M.

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Discovery of Clinically Approved Agents That Promote Suppression of Cystic Fibrosis Transmembrane Conductance Regulator Nonsense Mutations von Mutyam, Venkateshwar, Du, Ming, Xue, Xiaojiao, Keeling, Kim M, White, E Lucile, Bostwick, J Robert, Rasmussen, Lynn, Liu, Bo, Mazur, Marina, Hong, Jeong S, Falk Libby, Emily, Liang, Feng, Shang, Haibo, Mense, Martin, Suto, Mark J, Bedwell, David M, Rowe, Steven M

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