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

Chronic and acute modulator treatment restore wild-type-like activity and stability to the primary cystic fibrosis-causing CFTR variant

慢性及急性调节剂治疗可使导致囊性纤维化的原发性 CFTR 变体恢复到野生型水平的活性和稳定性。

Rodrat, Mayuree; Charlick, James N; Sheppard, David N

Clinical, functional and therapeutic evaluation of CFTR variant I507del

CFTR变异体I507del的临床、功能和治疗评估

Sharma, Neeraj; Starego, Katherine; Li, Hongyu; Kleist, Andrew B; Cox, Gabrielle; Havens, Madison; Neneh, Jalloh S; Parr, Alianna; Merlo, Nicholas A; Ren, Clement L; Vanscoy, Lori; Raraigh, Karen S; Merlo, Christian; Sheppard, David N; Cutting, Garry R

Managing an ageing cystic fibrosis population: challenges and priorities

管理老龄化囊性纤维化患者群体:挑战与优先事项

Frost, Freddy J; Peckham, Daniel G; Felton, Imogen C; Snowball, Joanna E; Gray, Robert D; Jones, Andrew M; Simmonds, Nicholas J; Lord, Robert W; Lip, Gregory Y H; Chandler, Hannah; Murphy, Kevin; Downey, Damian G; Sheppard, David N; Davies, Jane C; Bull, Jane; Sommer, Paula; Cupid, Belinda; Allen, Lucy; Duckers, Jamie

Native CFTR codon bias controls translation rate to balance off-pathway aggregation and channel function by conformational imprinting

天然CFTR密码子偏好性通过构象印记控制翻译速率,从而平衡非通路聚集和通道功能。

Yoon, Jae Seok; Li, Hongyu; Kim, Yonjung; Shishido, Hideki; Sheppard, David N; Lee, Min Goo; Skach, William R

In vitro platform to model the function of ionocytes in the human airway epithelium

用于模拟人呼吸道上皮细胞中离子细胞功能的体外平台

Vilà-González, Marta; Pinte, Laetitia; Fradique, Ricardo; Causa, Erika; Kool, Heleen; Rodrat, Mayuree; Morell, Carola Maria; Al-Thani, Maha; Porter, Linsey; Guo, Wenrui; Maeshima, Ruhina; Hart, Stephen L; McCaughan, Frank; Granata, Alessandra; Sheppard, David N; Floto, R Andres; Rawlins, Emma L; Cicuta, Pietro; Vallier, Ludovic

Two rare variants that affect the same amino acid in CFTR have distinct responses to ivacaftor.

影响 CFTR 中同一氨基酸的两种罕见变异对伊伐卡托的反应不同

Li Hongyu, Rodrat Mayuree, Al-Salmani Majid K, Veselu Diana-Florentina, Han Sangwoo T, Raraigh Karen S, Cutting Garry R, Sheppard David N

Experimental pharmacology in precision medicine

精准医学中的实验药理学

Urbaniak, Alicja; Thummel, Kenneth E; Alade, Ayoade N; Rettie, Allan E; Prasad, Bhagwat; De Nicolò, Amedeo; Martin, Jennifer H; Sheppard, David N; Jarvis, Michael F

Extracellular phosphate enhances the function of F508del-CFTR rescued by CFTR correctors

细胞外磷酸盐增强了CFTR矫正剂可挽救的F508del-CFTR的功能。

Saint-Criq, Vinciane; Wang, Yiting; Delpiano, Livia; Lin, JinHeng; Sheppard, David N; Gray, Michael A

Towards next generation therapies for cystic fibrosis: Folding, function and pharmacology of CFTR

迈向囊性纤维化的下一代疗法:CFTR 的折叠、功能和药理学

Bose, Samuel J; Krainer, Georg; Ng, Demi R S; Schenkel, Mathias; Shishido, Hideki; Yoon, Jae Seok; Haggie, Peter M; Schlierf, Michael; Sheppard, David N; Skach, William R

Anion carriers as potential treatments for cystic fibrosis: transport in cystic fibrosis cells, and additivity to channel-targeting drugs

阴离子载体作为囊性纤维化的潜在治疗方法:在囊性纤维化细胞中的转运以及与靶向通道药物的叠加效应

Li, Hongyu; Valkenier, Hennie; Thorne, Abigail G; Dias, Christopher M; Cooper, James A; Kieffer, Marion; Busschaert, Nathalie; Gale, Philip A; Sheppard, David N; Davis, Anthony P