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Items: 1 to 20 of 101

1.

S-Nitrosylation of CHIP Enhances F508Del-CFTR Maturation.

Zaman K, Knight J, Hussain F, Cao R, Estabrooks SK, Altawallbeh G, Holloway K, Jafri A, Sawczak V, Li Y, Getsy P, Sun F, Raffay T, Cotton C, Brodsky JL, Periasamy A, Lewis SJ, Gaston B.

Am J Respir Cell Mol Biol. 2019 Dec;61(6):765-775. doi: 10.1165/rcmb.2018-0314OC.

PMID:
31596601
2.

S-Nitrosothiols increases cystic fibrosis transmembrane regulator expression and maturation in the cell surface.

Zaman K, Bennett D, Fraser-Butler M, Greenberg Z, Getsy P, Sattar A, Smith L, Corey D, Sun F, Hunt J, Lewis SJ, Gaston B.

Biochem Biophys Res Commun. 2014 Jan 24;443(4):1257-62. doi: 10.1016/j.bbrc.2013.12.130. Epub 2014 Jan 3.

3.

Novel s-nitrosothiols have potential therapeutic uses for cystic fibrosis.

Zaman K, Fraser-Butler M, Bennett D.

Curr Pharm Des. 2013;19(19):3509-20. Review.

PMID:
23331028
4.

Novel Approaches for Potential Therapy of Cystic Fibrosis.

Sawczak V, Getsy P, Zaidi A, Sun F, Zaman K, Gaston B.

Curr Drug Targets. 2015;16(9):923-36. Review.

PMID:
25557257
5.

Augmentation of CFTR maturation by S-nitrosoglutathione reductase.

Zaman K, Sawczak V, Zaidi A, Butler M, Bennett D, Getsy P, Zeinomar M, Greenberg Z, Forbes M, Rehman S, Jyothikumar V, DeRonde K, Sattar A, Smith L, Corey D, Straub A, Sun F, Palmer L, Periasamy A, Randell S, Kelley TJ, Lewis SJ, Gaston B.

Am J Physiol Lung Cell Mol Physiol. 2016 Feb 1;310(3):L263-70. doi: 10.1152/ajplung.00269.2014. Epub 2015 Dec 4.

6.

S-nitrosylating agents: a novel class of compounds that increase cystic fibrosis transmembrane conductance regulator expression and maturation in epithelial cells.

Zaman K, Carraro S, Doherty J, Henderson EM, Lendermon E, Liu L, Verghese G, Zigler M, Ross M, Park E, Palmer LA, Doctor A, Stamler JS, Gaston B.

Mol Pharmacol. 2006 Oct;70(4):1435-42. Epub 2006 Jul 20.

PMID:
16857740
7.

A molecular switch in the scaffold NHERF1 enables misfolded CFTR to evade the peripheral quality control checkpoint.

Loureiro CA, Matos AM, Dias-Alves Â, Pereira JF, Uliyakina I, Barros P, Amaral MD, Matos P.

Sci Signal. 2015 May 19;8(377):ra48. doi: 10.1126/scisignal.aaa1580.

PMID:
25990958
8.

Augmentation of Cystic Fibrosis Transmembrane Conductance Regulator Function in Human Bronchial Epithelial Cells via SLC6A14-Dependent Amino Acid Uptake. Implications for Treatment of Cystic Fibrosis.

Ahmadi S, Wu YS, Li M, Ip W, Lloyd-Kuzik A, Di Paola M, Du K, Xia S, Lew A, Bozoky Z, Forman-Kay J, Bear CE, Gonska T.

Am J Respir Cell Mol Biol. 2019 Dec;61(6):755-764. doi: 10.1165/rcmb.2019-0094OC.

PMID:
31189070
9.

Increasing the Endoplasmic Reticulum Pool of the F508del Allele of the Cystic Fibrosis Transmembrane Conductance Regulator Leads to Greater Folding Correction by Small Molecule Therapeutics.

Chung WJ, Goeckeler-Fried JL, Havasi V, Chiang A, Rowe SM, Plyler ZE, Hong JS, Mazur M, Piazza GA, Keeton AB, White EL, Rasmussen L, Weissman AM, Denny RA, Brodsky JL, Sorscher EJ.

PLoS One. 2016 Oct 12;11(10):e0163615. doi: 10.1371/journal.pone.0163615. eCollection 2016.

10.

Reduced expression of Tis7/IFRD1 protein in murine and human cystic fibrosis airway epithelial cell models homozygous for the F508del-CFTR mutation.

Blanchard E, Marie S, Riffault L, Bonora M, Tabary O, Clement A, Jacquot J.

Biochem Biophys Res Commun. 2011 Aug 5;411(3):471-6. doi: 10.1016/j.bbrc.2011.06.104. Epub 2011 Jun 24.

PMID:
21723850
11.

MicroRNA-145 Antagonism Reverses TGF-β Inhibition of F508del CFTR Correction in Airway Epithelia.

Lutful Kabir F, Ambalavanan N, Liu G, Li P, Solomon GM, Lal CV, Mazur M, Halloran B, Szul T, Gerthoffer WT, Rowe SM, Harris WT.

Am J Respir Crit Care Med. 2018 Mar 1;197(5):632-643. doi: 10.1164/rccm.201704-0732OC.

12.
13.

Different SUMO paralogues determine the fate of wild-type and mutant CFTRs: biogenesis versus degradation.

Gong X, Liao Y, Ahner A, Larsen MB, Wang X, Bertrand CA, Frizzell RA.

Mol Biol Cell. 2019 Jan 1;30(1):4-16. doi: 10.1091/mbc.E18-04-0252. Epub 2018 Nov 7.

14.

The anion transporter SLC26A9 localizes to tight junctions and is degraded by the proteasome when co-expressed with F508del-CFTR.

Sato Y, Thomas DY, Hanrahan JW.

J Biol Chem. 2019 Nov 29;294(48):18269-18284. doi: 10.1074/jbc.RA119.010192. Epub 2019 Oct 23.

PMID:
31645438
15.

Rescue of functional F508del cystic fibrosis transmembrane conductance regulator by vasoactive intestinal peptide in the human nasal epithelial cell line JME/CF15.

Rafferty S, Alcolado N, Norez C, Chappe F, Pelzer S, Becq F, Chappe V.

J Pharmacol Exp Ther. 2009 Oct;331(1):2-13. doi: 10.1124/jpet.109.155341. Epub 2009 Jul 7.

PMID:
19584307
16.

Short-term consequences of F508del-CFTR thermal instability on CFTR-dependent transepithelial currents in human airway epithelial cells.

Froux L, Coraux C, Sage E, Becq F.

Sci Rep. 2019 Sep 24;9(1):13729. doi: 10.1038/s41598-019-50066-7.

17.

Two Small Molecules Restore Stability to a Subpopulation of the Cystic Fibrosis Transmembrane Conductance Regulator with the Predominant Disease-causing Mutation.

Meng X, Wang Y, Wang X, Wrennall JA, Rimington TL, Li H, Cai Z, Ford RC, Sheppard DN.

J Biol Chem. 2017 Mar 3;292(9):3706-3719. doi: 10.1074/jbc.M116.751537. Epub 2017 Jan 13.

18.

Roscovitine is a proteostasis regulator that corrects the trafficking defect of F508del-CFTR by a CDK-independent mechanism.

Norez C, Vandebrouck C, Bertrand J, Noel S, Durieu E, Oumata N, Galons H, Antigny F, Chatelier A, Bois P, Meijer L, Becq F.

Br J Pharmacol. 2014 Nov;171(21):4831-49. doi: 10.1111/bph.12859.

19.

Proteasome-dependent pharmacological rescue of cystic fibrosis transmembrane conductance regulator revealed by mutation of glycine 622.

Norez C, Bilan F, Kitzis A, Mettey Y, Becq F.

J Pharmacol Exp Ther. 2008 Apr;325(1):89-99. doi: 10.1124/jpet.107.134502. Epub 2008 Jan 29.

PMID:
18230692
20.

Partial rescue of F508del-cystic fibrosis transmembrane conductance regulator channel gating with modest improvement of protein processing, but not stability, by a dual-acting small molecule.

Liu J, Bihler H, Farinha CM, Awatade NT, Romão AM, Mercadante D, Cheng Y, Musisi I, Jantarajit W, Wang Y, Cai Z, Amaral MD, Mense M, Sheppard DN.

Br J Pharmacol. 2018 Apr;175(7):1017-1038. doi: 10.1111/bph.14141. Epub 2018 Feb 22.

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