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1
Tezacaftor–Ivacaftor in Residual-Function Heterozygotes with Cystic Fibrosis
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Tezacaftor–Ivacaftor in Residual-Function Heterozygotes with Cystic Fibrosis

The New England journal of medicine, 2017-11, Vol.377 (21), p.2024-2035 [Peer Reviewed Journal]

Copyright © 2017 Massachusetts Medical Society. All rights reserved. ;ISSN: 0028-4793 ;EISSN: 1533-4406 ;DOI: 10.1056/NEJMoa1709847 ;PMID: 29099333

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2
Tezacaftor–Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del
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Tezacaftor–Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del

The New England journal of medicine, 2017-11, Vol.377 (21), p.2013-2023 [Peer Reviewed Journal]

Copyright © 2017 Massachusetts Medical Society. All rights reserved. ;ISSN: 0028-4793 ;EISSN: 1533-4406 ;DOI: 10.1056/NEJMoa1709846 ;PMID: 29099344

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3
Blockade of IL-6 Trans signaling attenuates pulmonary fibrosis
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Blockade of IL-6 Trans signaling attenuates pulmonary fibrosis

The Journal of immunology (1950), 2014-10, Vol.193 (7), p.3755-3768 [Peer Reviewed Journal]

Copyright © 2014 by The American Association of Immunologists, Inc. ;Copyright © 2014 by The American Association of Immunologists, Inc. 2014 Copyright © 2014 by The American Association of Immunologists, Inc. ;ISSN: 0022-1767 ;EISSN: 1550-6606 ;DOI: 10.4049/jimmunol.1302470 ;PMID: 25172494

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4
Emerging role of HMGB1 in fibrotic diseases
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Emerging role of HMGB1 in fibrotic diseases

Journal of cellular and molecular medicine, 2014-12, Vol.18 (12), p.2331-2339 [Peer Reviewed Journal]

2014 The Authors. Journal of Cellular and Molecular Medicine published by John Wiley & Sons Ltd and Foundation for Cellular and Molecular Medicine. ;Copyright © 2014 John Wiley & Sons Ltd and Foundation for Cellular and Molecular Medicine ;2014 The Authors. Journal of Cellular and Molecular Medicine published by John Wiley & Sons Ltd and Foundation for Cellular and Molecular Medicine. 2014 ;ISSN: 1582-1838 ;EISSN: 1582-4934 ;DOI: 10.1111/jcmm.12419 ;PMID: 25284457

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5
Cystic fibrosis: a clinical view
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Article
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Cystic fibrosis: a clinical view

Cellular and molecular life sciences : CMLS, 2017-01, Vol.74 (1), p.129-140 [Peer Reviewed Journal]

Springer International Publishing 2016 ;Cellular and Molecular Life Sciences is a copyright of Springer, 2017. ;ISSN: 1420-682X ;EISSN: 1420-9071 ;DOI: 10.1007/s00018-016-2393-9 ;PMID: 27709245

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6
Efficacy and Safety of Ivacaftor in Patients Aged 6 to 11 Years with Cystic Fibrosis with a G551D Mutation
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Efficacy and Safety of Ivacaftor in Patients Aged 6 to 11 Years with Cystic Fibrosis with a G551D Mutation

American journal of respiratory and critical care medicine, 2013-06, Vol.187 (11), p.1219-1225 [Peer Reviewed Journal]

2014 INIST-CNRS ;Copyright American Thoracic Society Jun 1, 2013 ;Copyright © 2013 by the American Thoracic Society 2013 ;ISSN: 1073-449X ;EISSN: 1535-4970 ;DOI: 10.1164/rccm.201301-0153OC ;PMID: 23590265

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7
Spectrum of Fibrotic Lung Diseases
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Article
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Spectrum of Fibrotic Lung Diseases

The New England journal of medicine, 2020-09, Vol.383 (10), p.958-968 [Peer Reviewed Journal]

Copyright © 2020 Massachusetts Medical Society. All rights reserved. ;ISSN: 0028-4793 ;EISSN: 1533-4406 ;DOI: 10.1056/NEJMra2005230 ;PMID: 32877584

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8
Structure-guided combination therapy to potently improve the function of mutant CFTRs
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Article
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Structure-guided combination therapy to potently improve the function of mutant CFTRs

Nature medicine, 2018-11, Vol.24 (11), p.1732-1742 [Peer Reviewed Journal]

COPYRIGHT 2018 Nature Publishing Group ;COPYRIGHT 2018 Nature Publishing Group ;Copyright Nature Publishing Group Nov 2018 ;ISSN: 1078-8956 ;EISSN: 1546-170X ;DOI: 10.1038/s41591-018-0200-x ;PMID: 30297908

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9
A functional CFTR assay using primary cystic fibrosis intestinal organoids
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A functional CFTR assay using primary cystic fibrosis intestinal organoids

Nature medicine, 2013-07, Vol.19 (7), p.939-945 [Peer Reviewed Journal]

COPYRIGHT 2013 Nature Publishing Group ;COPYRIGHT 2013 Nature Publishing Group ;Copyright Nature Publishing Group Jul 2013 ;ISSN: 1078-8956 ;EISSN: 1546-170X ;DOI: 10.1038/nm.3201 ;PMID: 23727931

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10
Pulmonary fibrosis 4 months after COVID-19 is associated with severity of illness and blood leucocyte telomere length
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Pulmonary fibrosis 4 months after COVID-19 is associated with severity of illness and blood leucocyte telomere length

Thorax, 2021-12, Vol.76 (12), p.1242-1245 [Peer Reviewed Journal]

Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. ;2021 Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. http://creativecommons.org/licenses/by-nc/4.0/ This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/ . Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License. ;Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. 2021 ;ISSN: 0040-6376 ;EISSN: 1468-3296 ;DOI: 10.1136/thoraxjnl-2021-217031 ;PMID: 33927016

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11
SARS-CoV-2 infection triggers profibrotic macrophage responses and lung fibrosis
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Article
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SARS-CoV-2 infection triggers profibrotic macrophage responses and lung fibrosis

Cell, 2021-12, Vol.184 (26), p.6243-6261.e27 [Peer Reviewed Journal]

2021 ;Copyright © 2021. Published by Elsevier Inc. ;2021 Published by Elsevier Inc. 2021 ;ISSN: 0092-8674 ;EISSN: 1097-4172 ;DOI: 10.1016/j.cell.2021.11.033 ;PMID: 34914922

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12
Nutritional Status Improved in Cystic Fibrosis Patients with the G551D Mutation After Treatment with Ivacaftor
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Nutritional Status Improved in Cystic Fibrosis Patients with the G551D Mutation After Treatment with Ivacaftor

Digestive diseases and sciences, 2016-01, Vol.61 (1), p.198-207 [Peer Reviewed Journal]

Springer Science+Business Media New York 2015 ;COPYRIGHT 2016 Springer ;Springer Science+Business Media New York 2016 ;ISSN: 0163-2116 ;EISSN: 1573-2568 ;DOI: 10.1007/s10620-015-3834-2 ;PMID: 26250833 ;CODEN: DDSCDJ

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13
Pulmonary fibrosis: patterns and perpetrators
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Article
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Pulmonary fibrosis: patterns and perpetrators

The Journal of clinical investigation, 2012-08, Vol.122 (8), p.2756-2762 [Peer Reviewed Journal]

COPYRIGHT 2012 American Society for Clinical Investigation ;COPYRIGHT 2012 American Society for Clinical Investigation ;Copyright American Society for Clinical Investigation Aug 2012 ;Copyright © 2012, American Society for Clinical Investigation 2012 ;ISSN: 0021-9738 ;EISSN: 1558-8238 ;DOI: 10.1172/jci60323 ;PMID: 22850886

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14
Severe lung fibrosis requires an invasive fibroblast phenotype regulated by hyaluronan and CD44
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Severe lung fibrosis requires an invasive fibroblast phenotype regulated by hyaluronan and CD44

The Journal of experimental medicine, 2011-07, Vol.208 (7), p.1459-1471 [Peer Reviewed Journal]

2011 Li et al. 2011 ;ISSN: 0022-1007 ;EISSN: 1540-9538 ;DOI: 10.1084/jem.20102510 ;PMID: 21708929

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15
Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline
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Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline

American journal of respiratory and critical care medicine, 2022-05, Vol.205 (9), p.e18-e47 [Peer Reviewed Journal]

Copyright American Thoracic Society May 1, 2022 ;Distributed under a Creative Commons Attribution 4.0 International License ;ISSN: 1073-449X ;EISSN: 1535-4970 ;DOI: 10.1164/rccm.202202-0399st ;PMID: 35486072

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16
Origins of Cystic Fibrosis Lung Disease
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Article
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Origins of Cystic Fibrosis Lung Disease

The New England journal of medicine, 2015-01, Vol.372 (4), p.351-362 [Peer Reviewed Journal]

Copyright © 2015 Massachusetts Medical Society. All rights reserved. ;ISSN: 0028-4793 ;EISSN: 1533-4406 ;DOI: 10.1056/NEJMra1300109 ;PMID: 25607428

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17
Elexacaftor/Ivacaftor/Tezacaftor: First Approval
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Article
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Elexacaftor/Ivacaftor/Tezacaftor: First Approval

Drugs (New York, N.Y.), 2019-12, Vol.79 (18), p.2001-2007 [Peer Reviewed Journal]

Springer Nature Switzerland AG 2019 ;Copyright Springer Nature B.V. Dec 2019 ;ISSN: 0012-6667 ;EISSN: 1179-1950 ;DOI: 10.1007/s40265-019-01233-7 ;PMID: 31784874

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18
Capitalizing on the heterogeneous effects of CFTR nonsense and frameshift variants to inform therapeutic strategy for cystic fibrosis
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Capitalizing on the heterogeneous effects of CFTR nonsense and frameshift variants to inform therapeutic strategy for cystic fibrosis

PLoS genetics, 2018-11, Vol.14 (11), p.e1007723-e1007723 [Peer Reviewed Journal]

COPYRIGHT 2018 Public Library of Science ;COPYRIGHT 2018 Public Library of Science ;2018 Sharma et al. This is an open access article distributed under the terms of the Creative Commons Attribution License: http://creativecommons.org/licenses/by/4.0/ (the “License”), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License. ;2018 Sharma et al 2018 Sharma et al ;ISSN: 1553-7404 ;ISSN: 1553-7390 ;EISSN: 1553-7404 ;DOI: 10.1371/journal.pgen.1007723 ;PMID: 30444886

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19
Matrix metalloproteinases as therapeutic targets for idiopathic pulmonary fibrosis
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Matrix metalloproteinases as therapeutic targets for idiopathic pulmonary fibrosis

American journal of respiratory cell and molecular biology, 2015-11, Vol.53 (5), p.585-600 [Peer Reviewed Journal]

Copyright American Thoracic Society Nov 2015 ;Copyright © 2015 by the American Thoracic Society 2015 ;ISSN: 1044-1549 ;EISSN: 1535-4989 ;DOI: 10.1165/rcmb.2015-0020tr ;PMID: 26121236

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20
Deubiquitinase-targeting chimeras for targeted protein stabilization
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Deubiquitinase-targeting chimeras for targeted protein stabilization

Nature chemical biology, 2022-04, Vol.18 (4), p.412-421 [Peer Reviewed Journal]

2022. The Author(s), under exclusive licence to Springer Nature America, Inc. ;The Author(s), under exclusive licence to Springer Nature America, Inc. 2022. ;ISSN: 1552-4450 ;EISSN: 1552-4469 ;DOI: 10.1038/s41589-022-00971-2 ;PMID: 35210618

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