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Chronic ivacaftor treatment: getting F508del-CFTR into more trouble?

作者信息

Mall Marcus A, Sheppard David N

机构信息

Department of Translational Pulmonology, Translational Lung Research Center Heidelberg (TLRC), University of Heidelberg, Member of the German Center for Lung Research (DZL), Im Neuenheimer Feld 350, 69120, Heidelberg, Germany; Division of Pediatric Pulmonology & Cystic Fibrosis Center, University of Heidelberg, Member of the German Center for Lung Research (DZL), Heidelberg, Germany.

School of Physiology and Pharmacology, University of Bristol, Medical Sciences Building, University Walk, Bristol BS8 1TD, Bristol, UK.

出版信息

J Cyst Fibros. 2014 Dec;13(6):605-7. doi: 10.1016/j.jcf.2014.10.001. Epub 2014 Oct 16.

DOI:10.1016/j.jcf.2014.10.001
PMID:25458465
Abstract
摘要

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Chronic ivacaftor treatment: getting F508del-CFTR into more trouble?长期依伐卡托治疗：会让F508del-CFTR陷入更多困境吗？
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Lumacaftor alone and combined with ivacaftor: preclinical and clinical trial experience of F508del CFTR correction.鲁马卡托单独使用及与依伐卡托联合使用：F508del囊性纤维化跨膜传导调节因子校正的临床前及临床试验经验
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Can Cystic Fibrosis Patients Finally Catch a Breath With Lumacaftor/Ivacaftor?囊性纤维化患者能否最终借助鲁马卡托/依伐卡托顺畅呼吸？
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Intestinal Current Measurements Detect Activation of Mutant CFTR in Patients with Cystic Fibrosis with the G551D Mutation Treated with Ivacaftor.肠道电流测量可检测出接受依伐卡托治疗的携带G551D突变的囊性纤维化患者中突变型CFTR的激活情况。
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Effect of ivacaftor on CFTR forms with missense mutations associated with defects in protein processing or function.依伐卡托对 CFTR 错义突变相关蛋白加工或功能缺陷的影响。
J Cyst Fibros. 2014 Jan;13(1):29-36. doi: 10.1016/j.jcf.2013.06.008. Epub 2013 Jul 23.

引用本文的文献

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The potentiator ivacaftor is essential for pharmacological restoration of F508del-CFTR function and mucociliary clearance in cystic fibrosis.增效剂依伐卡托对于恢复囊性纤维化中F508del-CFTR功能和黏液纤毛清除功能的药理学作用至关重要。
JCI Insight. 2025 Apr 22;10(10). doi: 10.1172/jci.insight.187951. eCollection 2025 May 22.
2
The potentially beneficial central nervous system activity profile of ivacaftor and its metabolites.依伐卡托及其代谢物潜在有益的中枢神经系统活性概况。
ERJ Open Res. 2018 Mar 13;4(1). doi: 10.1183/23120541.00127-2017. eCollection 2018 Jan.
3
Mutation-specific downregulation of CFTR2 variants by gating potentiators.
门控增强剂特异性下调 CFTR2 变异体。
Hum Mol Genet. 2017 Dec 15;26(24):4873-4885. doi: 10.1093/hmg/ddx367.