Molecular Dynamics and Theratyping in Airway and Gut Organoids Reveal R352Q-CFTR Conductance Defect.
intestinal organoid
molecular dynamics simulations
nasal epithelial cells
personalized medicine
rare cystic fibrosis
Journal
American journal of respiratory cell and molecular biology
ISSN: 1535-4989
Titre abrégé: Am J Respir Cell Mol Biol
Pays: United States
ID NLM: 8917225
Informations de publication
Date de publication:
07 2022
07 2022
Historique:
pubmed:
27
4
2022
medline:
8
7
2022
entrez:
26
4
2022
Statut:
ppublish
Résumé
A significant challenge to making targeted cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies accessible to all individuals with cystic fibrosis (CF) are many mutations in the
Identifiants
pubmed: 35471184
doi: 10.1165/rcmb.2021-0337OC
pmc: PMC9273222
doi:
Substances chimiques
Aminophenols
0
CFTR protein, human
0
Chlorides
0
Cystic Fibrosis Transmembrane Conductance Regulator
126880-72-6
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
99-111Références
Biochemistry. 1999 Apr 27;38(17):5528-37
pubmed: 10220340
Eur Respir J. 2021 Jan 5;57(1):
pubmed: 32747394
Rare Dis. 2013 Nov 11;1:e27112
pubmed: 25003014
Mol Biol Cell. 2004 Jun;15(6):2684-96
pubmed: 15075371
Channels (Austin). 2018;12(1):284-290
pubmed: 30152709
JCI Insight. 2018 Jul 12;3(13):
pubmed: 29997283
J Gen Physiol. 2017 Mar 6;149(3):355-372
pubmed: 28242630
J Cyst Fibros. 2020 Mar;19(2):236-244
pubmed: 31678009
Front Pharmacol. 2020 Mar 17;11:295
pubmed: 32256364
J Biol Chem. 2021 Jan-Jun;296:100598
pubmed: 33781744
Science. 2015 Jan 23;347(6220):1260419
pubmed: 25613900
Cell Mol Life Sci. 2017 Jan;74(1):3-22
pubmed: 27717958
Ann Am Thorac Soc. 2021 Mar;18(3):433-441
pubmed: 33095038
Curr Opin Pharmacol. 2017 Jun;34:98-104
pubmed: 29073476
Sci Rep. 2019 Sep 17;9(1):13460
pubmed: 31530897
Lancet Respir Med. 2016 Aug;4(8):e37-e38
pubmed: 27377414
Eur Respir J. 2021 Jun 17;57(6):
pubmed: 33303536
J Pers Med. 2021 May 16;11(5):
pubmed: 34065744
Hum Mol Genet. 2017 Dec 15;26(24):4873-4885
pubmed: 29040544
J Cyst Fibros. 2014 Jan;13(1):29-36
pubmed: 23891399
J Cyst Fibros. 2019 Jan;18(1):22-34
pubmed: 29934203
Respir Res. 2013 Mar 28;14:38
pubmed: 23537407
Eur Respir J. 2018 Jun 7;51(6):
pubmed: 29563174
Am J Respir Cell Mol Biol. 2017 May;56(5):568-574
pubmed: 27983869
Biochem Pharmacol. 2013 Sep 1;86(5):612-9
pubmed: 23835419
Nat Med. 2013 Jul;19(7):939-45
pubmed: 23727931
Lancet Respir Med. 2020 Jan;8(1):65-124
pubmed: 31570318
Cell. 2015 Oct 22;163(3):724-33
pubmed: 26496611
EMBO Mol Med. 2017 Sep;9(9):1224-1243
pubmed: 28667089
Sci Rep. 2021 Oct 6;11(1):19810
pubmed: 34615919
JCI Insight. 2018 Jul 26;3(14):
pubmed: 30046002
Am J Respir Cell Mol Biol. 2002 Oct;27(4):503-10
pubmed: 12356585
J Clin Invest. 1993 Apr;91(4):1590-7
pubmed: 8473502
iScience. 2021 Dec 31;25(1):103710
pubmed: 35072004
Am J Respir Crit Care Med. 2019 Jan 1;199(1):123-126
pubmed: 30326728
Cells. 2020 Sep 21;9(9):
pubmed: 32967385
Mol Biol Cell. 2013 Oct;24(19):3016-24
pubmed: 23924900
Proc Natl Acad Sci U S A. 2018 Dec 11;115(50):12757-12762
pubmed: 30459277
Am J Respir Cell Mol Biol. 2010 Mar;42(3):363-72
pubmed: 19502384
Cell Rep. 2019 Feb 12;26(7):1701-1708.e3
pubmed: 30759382
Front Pharmacol. 2018 Dec 19;9:1490
pubmed: 30618775
Cell Mol Life Sci. 2017 Jan;74(1):93-115
pubmed: 27714410
Nat Rev Dis Primers. 2015 May 14;1:15010
pubmed: 27189798
Sci Transl Med. 2016 Jun 22;8(344):344ra84
pubmed: 27334259
Chem Rev. 2019 Jul 10;119(13):7737-7832
pubmed: 31246417
Proc Natl Acad Sci U S A. 2011 Nov 15;108(46):18843-8
pubmed: 21976485
Sci Rep. 2017 Dec 21;7(1):17971
pubmed: 29269735
J Cyst Fibros. 2021 Mar;20(2):364-371
pubmed: 33414087
Sci Rep. 2017 Aug 7;7(1):7375
pubmed: 28785019
J Cyst Fibros. 2021 Sep;20(5):895-898
pubmed: 33775603
Nat Chem Biol. 2013 Jul;9(7):444-54
pubmed: 23666117
J Biol Chem. 2013 Jul 12;288(28):20758-67
pubmed: 23709221
J Membr Biol. 2008 Mar;222(2):91-106
pubmed: 18421494
Biochim Biophys Acta. 2010 Nov;1802(11):1062-9
pubmed: 20696241