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

STAT3-Dependent Regulation of CFTR and Ciliogenesis Is Essential for Mucociliary Clearance and Innate Airway Defense in Hyper-IgE Syndrome

STAT3依赖的CFTR和纤毛发生调控对于高IgE综合征中的黏液纤毛清除和先天性气道防御至关重要

Sun, Ling; Walls, Samantha A; Dang, Hong; Quinney, Nancy L; Sears, Patrick R; Sadritabrizi, Taraneh; Hasegawa, Koichi; Okuda, Kenichi; Asakura, Takanori; Chang, Xiuya; Zheng, Meiqi; Mikami, Yu; Dizmond, Felicia U; Danilova, Daniela; Zhou, Lynn; Deshmukh, Anshulika; Cholon, Deborah M; Radicioni, Giorgia; Rogers, Troy D; Kissner, William J; Markovetz, Matthew R; Guhr Lee, Tara N; Gutay, Mark I; Esther, Charles R Jr; Chua, Michael; Grubb, Barbara R; Ehre, Camille; Kesimer, Mehmet; Hill, David B; Ostrowski, Lawrence E; Button, Brian; Gentzsch, Martina; Robinson, Chevalia; Olivier, Kenneth N; Freeman, Alexandra F; Randell, Scott H; Vladar, Eszter; O'Neal, Wanda K; Boucher, Richard C; Chen, Gang

Implications for cystic fibrosis therapy: Potentiator icenticaftor is superior to ivacaftor in improving function and maintaining stability of F508del CFTR

对囊性纤维化治疗的意义:增强剂icenticaftor在改善F508del CFTR的功能和维持其稳定性方面优于ivacaftor。

Cholon, Deborah M; Aleksandrov, Luba A; Quinney, Nancy L; Boyles, Susan E; Jensen, Timothy J; Aleksandrov, Andrei A; Gentzsch, Martina

Cystic fibrosis airway inflammation enables elexacaftor/tezacaftor/ivacaftor-mediated rescue of N1303K CFTR mutation

囊性纤维化气道炎症可使 elexacaftor/tezacaftor/ivacaftor 介导的 N1303K CFTR 突变得到挽救

Gentzsch, Martina; Baker, Brooke; Cholon, Deborah M; Kam, Charissa W; McKinzie, Cameron J; Despotes, Katherine A; Boyles, Susan E; Quinney, Nancy L; Esther, Charles R Jr; Ribeiro, Carla M P

A Novel Co-Culture Model Reveals Enhanced CFTR Rescue in Primary Cystic Fibrosis Airway Epithelial Cultures with Persistent Pseudomonas aeruginosa Infection

一种新型共培养模型揭示了在持续性铜绿假单胞菌感染的原发性囊性纤维化气道上皮培养物中增强的 CFTR 拯救

Deborah M Cholon, Matthew A Greenwald, Matthew G Higgs, Nancy L Quinney, Susan E Boyles, Suzanne L Meinig, John T Minges, Ashlesha Chaubal, Robert Tarran, Carla M P Ribeiro, Matthew C Wolfgang, Martina Gentzsch

Small-molecule eRF3a degraders rescue CFTR nonsense mutations by promoting premature termination codon readthrough

小分子 eRF3a 降解剂通过促进提前终止密码子读通来挽救 CFTR 无义突变

Rhianna E Lee, Catherine A Lewis, Lihua He, Emily C Bulik-Sullivan, Samuel C Gallant, Teresa M Mascenik, Hong Dang, Deborah M Cholon, Martina Gentzsch, Lisa C Morton, John T Minges, Jonathan W Theile, Neil A Castle, Michael R Knowles, Adam J Kimple, Scott H Randell

Established and novel human translational models to advance cystic fibrosis research, drug discovery, and optimize CFTR-targeting therapeutics

已建立和新型的人体转化模型,用于推进囊性纤维化研究、药物发现和优化CFTR靶向疗法

Cholon, Deborah M; Gentzsch, Martina

A small-molecule inhibitor and degrader of the RNF5 ubiquitin ligase

RNF5 泛素连接酶的小分子抑制剂和降解剂

Jingjing Ruan, Dongdong Liang, Wenjing Yan, Yongwang Zhong, Daniel C Talley, Ganesha Rai, Dingyin Tao, Christopher A LeClair, Anton Simeonov, Yinghua Zhang, Feihu Chen, Nancy L Quinney, Susan E Boyles, Deborah M Cholon, Martina Gentzsch, Mark J Henderson, Fengtian Xue, Shengyun Fang

Airway Epithelial Inflammation In Vitro Augments the Rescue of Mutant CFTR by Current CFTR Modulator Therapies

体外气道上皮炎症增强了当前 CFTR 调节剂疗法对突变 CFTR 的挽救作用

Martina Gentzsch, Deborah M Cholon, Nancy L Quinney, Mary E B Martino, John T Minges, Susan E Boyles, Tara N Guhr Lee, Charles R Esther Jr, Carla M P Ribeiro

DNAJB12 and Hsp70 triage arrested intermediates of N1303K-CFTR for endoplasmic reticulum-associated autophagy

DNAJB12 和 Hsp70 分类阻断 N1303K-CFTR 中间体,用于内质网相关自噬

Lihua He, Andrew S Kennedy, Scott Houck, Andrei Aleksandrov, Nancy L Quinney, Alexandra Cyr-Scully, Deborah M Cholon, Martina Gentzsch, Scott H Randell, Hong Yu Ren, Douglas M Cyr

Personalised medicine for non-classic cystic fibrosis resulting from rare CFTR mutations

针对由罕见CFTR基因突变引起的非典型囊性纤维化的个性化医疗

McCravy, Matthew S; Quinney, Nancy L; Cholon, Deborah M; Boyles, Susan E; Jensen, Timothy J; Aleksandrov, Andrei A; Donaldson, Scott H; Noone, Peadar G; Gentzsch, Martina