Both the autophagy and proteasomal pathways facilitate the Ubp3p-dependent depletion of a subset of translation and RNA turnover factors during nitrogen starvation in Saccharomyces...

por Kelly, Shane P., Bedwell, David M.
Publicado 2015

Finding sense in the context

por Keeling, Kim M, Bedwell, David M
Publicado 2020

Attenuation of Nonsense-Mediated mRNA Decay Enhances In Vivo Nonsense Suppression

por 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.
Publicado 2013

The synthetic aminoglycoside ELX-02 induces readthrough of G550X-CFTR producing superfunctional protein that can be further enhanced by CFTR modulators

por 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.
Publicado 2023

Mutation-Directed Therapeutics for Neurofibromatosis Type I

por 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
Publicado 2020

Combining nonsense mutation suppression therapy with nonsense-mediated decay inhibition in neurofibromatosis type 1

por Osum, Sara H., Oribamise, Eunice I., Corbière, Stanislas M.A.S., Taisto, Mandy, Jubenville, Tyler, Coutts, Alex, Kirstein, Mark N., Fisher, James, Moertel, Christopher, Du, Ming, Bedwell, David, Largaespada, David A., Watson, Adrienne L.
Publicado 2023

Prime editing-mediated correction of the CFTR W1282X mutation in iPSCs and derived airway epithelial cells

por Li, Chao, Liu, Zhong, Anderson, Justin, Liu, Zhongyu, Tang, Liping, Li, Yao, Peng, Ning, Chen, Jianguo, Liu, Xueming, Fu, Lianwu, Townes, Tim M., Rowe, Steven M., Bedwell, David M., Guimbellot, Jennifer, Zhao, Rui
Publicado 2023

Mice with missense and nonsense NF1 mutations display divergent phenotypes compared with human neurofibromatosis type I

por Li, Kairong, Turner, Ashley N., Chen, Min, Brosius, Stephanie N., Schoeb, Trenton R., Messiaen, Ludwine M., Bedwell, David M., Zinn, Kurt R., Anastasaki, Corina, Gutmann, David H., Korf, Bruce R., Kesterson, Robert A.
Publicado 2016

Triamterene Functions as an Effective Nonsense Suppression Agent for MPS I-H (Hurler Syndrome)

por 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.
Publicado 2023

Characterization of Defects in Ion Transport and Tissue Development in Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)-Knockout Rats

por Tuggle, Katherine L., Birket, Susan E., Cui, Xiaoxia, Hong, Jeong, Warren, Joe, Reid, Lara, Chambers, Andre, Ji, Diana, Gamber, Kevin, Chu, Kengyeh K., Tearney, Guillermo, Tang, Li Ping, Fortenberry, James A., Du, Ming, Cadillac, Joan M., Bedwell, David M., Rowe, Steven M., Sorscher, Eric J., Fanucchi, Michelle V.
Publicado 2014

A small molecule that induces translational readthrough of CFTR nonsense mutations by eRF1 depletion

por 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.
Publicado 2021