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AJRCCM: 100-Year Anniversary. Progress along the Pathway of Discovery Leading to Treatment and Cure of Cystic Fibrosis.

作者信息

Ramsey Bonnie W, Welsh Michael J

机构信息

1 Department of Pediatrics University of Washington School of Medicine Seattle, Washington.

2 Center for Clinical and Translational Research Seattle Children's Research Institute Seattle, Washington.

出版信息

Am J Respir Crit Care Med. 2017 May 1;195(9):1092-1099. doi: 10.1164/rccm.201702-0266ED.

DOI:10.1164/rccm.201702-0266ED
PMID:28459323
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5439020/
Abstract
摘要

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本文引用的文献

1
Diagnosis of Cystic Fibrosis: Consensus Guidelines from the Cystic Fibrosis Foundation.囊性纤维化的诊断：来自囊性纤维化基金会的共识指南
J Pediatr. 2017 Feb;181S:S4-S15.e1. doi: 10.1016/j.jpeds.2016.09.064.
2
New horizons for cystic fibrosis treatment.囊性纤维化治疗的新视野。
Pharmacol Ther. 2017 Feb;170:205-211. doi: 10.1016/j.pharmthera.2016.11.009. Epub 2016 Dec 1.
3
Atomic Structure of the Cystic Fibrosis Transmembrane Conductance Regulator.囊性纤维化跨膜电导调节子的原子结构。
Cell. 2016 Dec 1;167(6):1586-1597.e9. doi: 10.1016/j.cell.2016.11.014.
4
Early Lung Disease in Infants and Preschool Children with Cystic Fibrosis. What Have We Learned and What Should We Do about It?囊性纤维化婴幼儿及学龄前儿童的早期肺部疾病。我们学到了什么，又该如何应对？
Am J Respir Crit Care Med. 2017 Jun 15;195(12):1567-1575. doi: 10.1164/rccm.201606-1107CI.
5
Lumacaftor/Ivacaftor Treatment of Patients with Cystic Fibrosis Heterozygous for F508del-CFTR.鲁马卡托/依伐卡托治疗F508del-CFTR杂合型囊性纤维化患者
Ann Am Thorac Soc. 2017 Feb;14(2):213-219. doi: 10.1513/AnnalsATS.201609-689OC.
6
Architecture and functional properties of the CFTR channel pore.囊性纤维化跨膜传导调节因子（CFTR）通道孔的结构与功能特性。
Cell Mol Life Sci. 2017 Jan;74(1):67-83. doi: 10.1007/s00018-016-2389-5. Epub 2016 Oct 3.
7
Genetic medicines for CF: Hype versus reality.用于囊性纤维化的基因药物：炒作与现实
Pediatr Pulmonol. 2016 Oct;51(S44):S5-S17. doi: 10.1002/ppul.23543.
8
Discovery of Clinically Approved Agents That Promote Suppression of Cystic Fibrosis Transmembrane Conductance Regulator Nonsense Mutations.发现可促进抑制囊性纤维化跨膜传导调节因子无义突变的临床批准药物。
Am J Respir Crit Care Med. 2016 Nov 1;194(9):1092-1103. doi: 10.1164/rccm.201601-0154OC.
9
Airway acidification initiates host defense abnormalities in cystic fibrosis mice.气道酸化引发囊性纤维化小鼠的宿主防御异常。
Science. 2016 Jan 29;351(6272):503-7. doi: 10.1126/science.aad5589.
10
From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations.从囊性纤维化跨膜传导调节因子生物学走向联合药物治疗：囊性纤维化突变的扩展分类
Mol Biol Cell. 2016 Feb 1;27(3):424-33. doi: 10.1091/mbc.E14-04-0935.