日期:
2020 年 — 2026 年
2020
2021
2022
2023
2024
2025
2026
影响因子:

Identity, functional consequences, and context effects of amino acids inserted during suppression of CFTR nonsense mutations

CFTR无义突变抑制过程中插入的氨基酸的身份、功能后果和背景效应

Thrasher, Kari; Chen, Jianguo; Fu, Lianwu; Benson, Dominique; Ekstrom, Jenna; Hargett, Audra A; Renfrow, Matthew B; Rowe, Steven M; Keeling, Kim M; Bedwell, David M

Identification of small molecules that enhance aminoglycoside-mediated suppression of CFTR and NF1 nonsense mutations.

鉴定能增强氨基糖苷类药物对 CFTR 和 NF1 无义突变抑制作用的小分子。

Sammons Joshua, Chen Jianguo, Thrasher Kari, Fu Lianwu, Du Ming, Wen Hui, Bostwick J Robert, Vinson Paige, Moukha-Chafiq Omar, Augelli-Szafran Corinne, Keeling Kim M, Wallis Deeann, Kesterson Robert A, Rowe Steven M, Bedwell David M

Finding sense in the context

在语境中寻找意义

Keeling, Kim M; Bedwell, David M

Mutation-Directed Therapeutics for Neurofibromatosis Type I

针对I型神经纤维瘤病的突变导向疗法

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

Ataluren stimulates ribosomal selection of near-cognate tRNAs to promote nonsense suppression

阿塔卢伦刺激核糖体选择近同源tRNA,从而促进无义突变抑制。

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

Marked repression of CFTR mRNA in the transgenic Cftr(tm1kth) mouse model

转基因 Cftr(tm1kth) 小鼠模型中 CFTR mRNA 显著抑制

Zhang, Shaoyan; Ranganath, Neel K; Skinner, Daniel; Bedwell, David M; Buckley-Lanier, Jessica A; Sorscher, Eric J; Woodworth, Bradford A

Synthetic aminoglycosides efficiently suppress cystic fibrosis transmembrane conductance regulator nonsense mutations and are enhanced by ivacaftor

合成氨基糖苷类抗生素能有效抑制囊性纤维化跨膜传导调节因子无义突变,且其疗效可被伊伐卡托增强。

Xue, Xiaojiao; Mutyam, Venkateshwar; Tang, Liping; Biswas, Silpak; Du, Ming; Jackson, Laura A; Dai, Yanying; Belakhov, Valery; Shalev, Moran; Chen, Fuquan; Schacht, Jochen; J Bridges, Robert; Baasov, Timor; Hong, Jeong; Bedwell, David M; Rowe, Steven M

Long-term nonsense suppression therapy moderates MPS I-H disease progression

长期无义突变抑制疗法可延缓MPS IH疾病进展。

Gunn, Gwen; Dai, Yanying; Du, Ming; Belakhov, Valery; Kandasamy, Jeyakumar; Schoeb, Trenton R; Baasov, Timor; Bedwell, David M; Keeling, Kim M

Characterization of defects in ion transport and tissue development in cystic fibrosis transmembrane conductance regulator (CFTR)-knockout rats.

囊性纤维化跨膜传导调节因子 (CFTR) 敲除大鼠离子转运和组织发育缺陷的特征分析

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

Heterozygosity for the F508del mutation in the cystic fibrosis transmembrane conductance regulator anion channel attenuates influenza severity

囊性纤维化跨膜电导调节阴离子通道中F508del突变的杂合性可减轻流感严重程度。

Aeffner, Famke; Abdulrahman, Basant; Hickman-Davis, Judy M; Janssen, Paul M; Amer, Amal; Bedwell, David M; Sorscher, Eric J; Davis, Ian C