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Items: 1 to 50 of 154

1.

Author Correction: Airway surface liquid acidification initiates host defense abnormalities in Cystic Fibrosis.

Simonin J, Bille E, Crambert G, Noel S, Dreano E, Edwards A, Hatton A, Pranke I, Villeret B, Cottart CH, Vrel JP, Urbach V, Baatallah N, Hinzpeter A, Golec A, Touqui L, Nassif X, Galietta LJV, Planelles G, Sallenave JM, Edelman A, Sermet-Gaudelus I.

Sci Rep. 2019 Nov 21;9(1):17535. doi: 10.1038/s41598-019-54253-4.

2.

TRPV4 and purinergic receptor signalling pathways are separately linked in airway epithelia to CFTR and TMEM16A chloride channels.

Genovese M, Borrelli A, Venturini A, Guidone D, Caci E, Viscido G, Gambardella G, di Bernardo D, Scudieri P, Galietta LJV.

J Physiol. 2019 Dec;597(24):5859-5878. doi: 10.1113/JP278784. Epub 2019 Nov 12.

PMID:
31622498
3.

ANO4 (Anoctamin 4) Is a Novel Marker of Zona Glomerulosa That Regulates Stimulated Aldosterone Secretion.

Maniero C, Scudieri P, Haris Shaikh L, Zhao W, Gurnell M, Galietta LJV, Brown MJ.

Hypertension. 2019 Nov;74(5):1152-1159. doi: 10.1161/HYPERTENSIONAHA.119.13287. Epub 2019 Sep 30.

4.

Corrigendum: Normal Calcium-Activated Anion Secretion in a Mouse Selectively Lacking TMEM16A in Intestinal Epithelium.

Vega G, Guequén A, Johansson MEV, Arike L, Martínez-Abad B, Nyström EEL, Scudieri P, Pedemonte N, Millar-Büchner P, Philp AR, Galietta LJ, Hansson GC, Flores CA.

Front Physiol. 2019 Aug 27;10:1040. doi: 10.3389/fphys.2019.01040. eCollection 2019.

5.

An overview on chemical structures as ΔF508-CFTR correctors.

Spanò V, Montalbano A, Carbone A, Scudieri P, Galietta LJV, Barraja P.

Eur J Med Chem. 2019 Oct 15;180:430-448. doi: 10.1016/j.ejmech.2019.07.037. Epub 2019 Jul 15. Review.

PMID:
31326599
6.

Furocoumarins as multi-target agents in the treatment of cystic fibrosis.

Carbone A, Montalbano A, Spanò V, Musante I, Galietta LJV, Barraja P.

Eur J Med Chem. 2019 Oct 15;180:283-290. doi: 10.1016/j.ejmech.2019.07.025. Epub 2019 Jul 10. Review.

PMID:
31319264
7.

Bioactive Thymosin Alpha-1 Does Not Influence F508del-CFTR Maturation and Activity.

Armirotti A, Tomati V, Matthes E, Veit G, Cholon DM, Phuan PW, Braccia C, Guidone D, Gentzsch M, Lukacs GL, Verkman AS, Galietta LJV, Hanrahan JW, Pedemonte N.

Sci Rep. 2019 Jul 16;9(1):10310. doi: 10.1038/s41598-019-46639-1.

8.

Normal Calcium-Activated Anion Secretion in a Mouse Selectively Lacking TMEM16A in Intestinal Epithelium.

Vega G, Guequén A, Johansson MEV, Arike L, Martínez-Abad B, Nyström EEL, Scudieri P, Pedemonte N, Millar-Büchner P, Philp AR, Galietta LJ, Hansson GC, Flores CA.

Front Physiol. 2019 Jun 13;10:694. doi: 10.3389/fphys.2019.00694. eCollection 2019. Erratum in: Front Physiol. 2019 Aug 27;10:1040.

9.

High-Throughput Screening for Modulators of CFTR Activity Based on Genetically Engineered Cystic Fibrosis Disease-Specific iPSCs.

Merkert S, Schubert M, Olmer R, Engels L, Radetzki S, Veltman M, Scholte BJ, Zöllner J, Pedemonte N, Galietta LJV, von Kries JP, Martin U.

Stem Cell Reports. 2019 Jun 11;12(6):1389-1403. doi: 10.1016/j.stemcr.2019.04.014. Epub 2019 May 9.

10.

Airway surface liquid acidification initiates host defense abnormalities in Cystic Fibrosis.

Simonin J, Bille E, Crambert G, Noel S, Dreano E, Edwards A, Hatton A, Pranke I, Villeret B, Cottart CH, Vrel JP, Urbach V, Baatallah N, Hinzpeter A, Golec A, Touqui L, Nassif X, Galietta LJV, Planelles G, Sallenave JM, Edelman A, Sermet-Gaudelus I.

Sci Rep. 2019 Apr 24;9(1):6516. doi: 10.1038/s41598-019-42751-4. Erratum in: Sci Rep. 2019 Nov 21;9(1):17535.

11.

Thymosin α-1 does not correct F508del-CFTR in cystic fibrosis airway epithelia.

Tomati V, Caci E, Ferrera L, Pesce E, Sondo E, Cholon DM, Quinney NL, Boyles SE, Armirotti A, Ravazzolo R, Galietta LJ, Gentzsch M, Pedemonte N.

JCI Insight. 2019 Apr 4;4(7). pii: 128935. doi: 10.1172/jci.insight.128935. eCollection 2019 Apr 4. No abstract available.

12.

Peripheral localization of the epithelial sodium channel in the apical membrane of bronchial epithelial cells.

Musante I, Scudieri P, Venturini A, Guidone D, Caci E, Castellani S, Conese M, Galietta LJV.

Exp Physiol. 2019 Jun;104(6):866-875. doi: 10.1113/EP087590. Epub 2019 Apr 8.

PMID:
30924990
13.

Two CFTR mutations within codon 970 differently impact on the chloride channel functionality.

Amato F, Scudieri P, Musante I, Tomati V, Caci E, Comegna M, Maietta S, Manzoni F, Di Lullo AM, De Wachter E, Vanderhelst E, Terlizzi V, Braggion C, Castaldo G, Galietta LJV.

Hum Mutat. 2019 Jun;40(6):742-748. doi: 10.1002/humu.23741. Epub 2019 Mar 28.

PMID:
30851139
14.

Airway microenvironment alterations and pathogen growth in cystic fibrosis.

Rossi GA, Morelli P, Galietta LJ, Colin AA.

Pediatr Pulmonol. 2019 Apr;54(4):497-506. doi: 10.1002/ppul.24246. Epub 2019 Jan 8. Review.

PMID:
30620146
15.

The Autophagy Inhibitor Spautin-1 Antagonizes Rescue of Mutant CFTR Through an Autophagy-Independent and USP13-Mediated Mechanism.

Pesce E, Sondo E, Ferrera L, Tomati V, Caci E, Scudieri P, Musante I, Renda M, Baatallah N, Servel N, Hinzpeter A, di Bernardo D, Pedemonte N, Galietta LJV.

Front Pharmacol. 2018 Dec 13;9:1464. doi: 10.3389/fphar.2018.01464. eCollection 2018.

16.

Increased expression of ATP12A proton pump in cystic fibrosis airways.

Scudieri P, Musante I, Caci E, Venturini A, Morelli P, Walter C, Tosi D, Palleschi A, Martin-Vasallo P, Sermet-Gaudelus I, Planelles G, Crambert G, Galietta LJ.

JCI Insight. 2018 Oct 18;3(20). pii: 123616. doi: 10.1172/jci.insight.123616.

17.

Combination potentiator ('co-potentiator') therapy for CF caused by CFTR mutants, including N1303K, that are poorly responsive to single potentiators.

Phuan PW, Son JH, Tan JA, Li C, Musante I, Zlock L, Nielson DW, Finkbeiner WE, Kurth MJ, Galietta LJ, Haggie PM, Verkman AS.

J Cyst Fibros. 2018 Sep;17(5):595-606. doi: 10.1016/j.jcf.2018.05.010. Epub 2018 Jun 12.

18.

A European regulatory perspective on cystic fibrosis: current treatments, trends in drug development and translational challenges for CFTR modulators.

Ponzano S, Nigrelli G, Fregonese L, Eichler I, Bertozzi F, Bandiera T, Galietta LJV, Papaluca M.

Eur Respir Rev. 2018 Apr 13;27(148). pii: 170124. doi: 10.1183/16000617.0124-2017. Print 2018 Jun 30. Review.

19.

Thymosin α-1 does not correct F508del-CFTR in cystic fibrosis airway epithelia.

Tomati V, Caci E, Ferrera L, Pesce E, Sondo E, Cholon DM, Quinney NL, Boyles SE, Armirotti A, Ravazzolo R, Galietta LJ, Gentzsch M, Pedemonte N.

JCI Insight. 2018 Feb 8;3(3). pii: 98699. doi: 10.1172/jci.insight.98699. eCollection 2018 Feb 8. Erratum in: JCI Insight. 2019 Apr 4;4(7):.

20.

Speeding Up the Identification of Cystic Fibrosis Transmembrane Conductance Regulator-Targeted Drugs: An Approach Based on Bioinformatics Strategies and Surface Plasmon Resonance.

Rusnati M, Sala D, Orro A, Bugatti A, Trombetti G, Cichero E, Urbinati C, Di Somma M, Millo E, Galietta LJV, Milanesi L, Fossa P, D'Ursi P.

Molecules. 2018 Jan 8;23(1). pii: E120. doi: 10.3390/molecules23010120.

21.

Synthesis and biological evaluation of novel thiazole- VX-809 hybrid derivatives as F508del correctors by QSAR-based filtering tools.

Liessi N, Cichero E, Pesce E, Arkel M, Salis A, Tomati V, Paccagnella M, Damonte G, Tasso B, Galietta LJV, Pedemonte N, Fossa P, Millo E.

Eur J Med Chem. 2018 Jan 20;144:179-200. doi: 10.1016/j.ejmech.2017.12.030. Epub 2017 Dec 8.

PMID:
29272749
22.

High-throughput screening identifies FAU protein as a regulator of mutant cystic fibrosis transmembrane conductance regulator channel.

Tomati V, Pesce E, Caci E, Sondo E, Scudieri P, Marini M, Amato F, Castaldo G, Ravazzolo R, Galietta LJV, Pedemonte N.

J Biol Chem. 2018 Jan 26;293(4):1203-1217. doi: 10.1074/jbc.M117.816595. Epub 2017 Nov 20.

23.

Bypassing CFTR dysfunction in cystic fibrosis with alternative pathways for anion transport.

Li H, Salomon JJ, Sheppard DN, Mall MA, Galietta LJ.

Curr Opin Pharmacol. 2017 Jun;34:91-97. doi: 10.1016/j.coph.2017.10.002. Epub 2017 Oct 21. Review.

PMID:
29065356
24.

Substituted 2-Acylaminocycloalkylthiophene-3-carboxylic Acid Arylamides as Inhibitors of the Calcium-Activated Chloride Channel Transmembrane Protein 16A (TMEM16A).

Truong EC, Phuan PW, Reggi AL, Ferrera L, Galietta LJV, Levy SE, Moises AC, Cil O, Diez-Cecilia E, Lee S, Verkman AS, Anderson MO.

J Med Chem. 2017 Jun 8;60(11):4626-4635. doi: 10.1021/acs.jmedchem.7b00020. Epub 2017 May 24.

25.

Intermolecular Interactions in the TMEM16A Dimer Controlling Channel Activity.

Scudieri P, Musante I, Gianotti A, Moran O, Galietta LJ.

Sci Rep. 2016 Dec 8;6:38788. doi: 10.1038/srep38788.

26.

Phenylquinoxalinone CFTR activator as potential prosecretory therapy for constipation.

Cil O, Phuan PW, Son JH, Zhu JS, Ku CK, Tabib NA, Teuthorn AP, Ferrera L, Zachos NC, Lin R, Galietta LJV, Donowitz M, Kurth MJ, Verkman AS.

Transl Res. 2017 Apr;182:14-26.e4. doi: 10.1016/j.trsl.2016.10.003. Epub 2016 Oct 15.

27.

TMEM16 Proteins: Membrane Channels with Unusual Pores.

Galietta LJV.

Biophys J. 2016 Nov 1;111(9):1821-1822. doi: 10.1016/j.bpj.2016.09.033. No abstract available.

28.

Goblet Cell Hyperplasia Requires High Bicarbonate Transport To Support Mucin Release.

Gorrieri G, Scudieri P, Caci E, Schiavon M, Tomati V, Sirci F, Napolitano F, Carrella D, Gianotti A, Musante I, Favia M, Casavola V, Guerra L, Rea F, Ravazzolo R, Di Bernardo D, Galietta LJ.

Sci Rep. 2016 Oct 27;6:36016. doi: 10.1038/srep36016.

29.

CFTR pharmacology.

Zegarra-Moran O, Galietta LJ.

Cell Mol Life Sci. 2017 Jan;74(1):117-128. doi: 10.1007/s00018-016-2392-x. Epub 2016 Oct 4. Review.

PMID:
27704174
30.

Correction: High-throughput screening for modulators of ACVR1 transcription: discovery of potential therapeutics for fibrodysplasia ossificans progressiva.

Cappato S, Tonachini L, Giacopelli F, Tirone M, Galietta LJ, Sormani M, Giovenzana A, Spinelli AE, Canciani B, Brunelli S, Ravazzolo R, Bocciardi R.

Dis Model Mech. 2016 Sep 1;9(9):1067. doi: 10.1242/dmm.027573. No abstract available.

31.

The ubiquitin ligase tripartite-motif-protein 32 is induced in Duchenne muscular dystrophy.

Assereto S, Piccirillo R, Baratto S, Scudieri P, Fiorillo C, Massacesi M, Traverso M, Galietta LJ, Bruno C, Minetti C, Zara F, Gazzerro E.

Lab Invest. 2016 Aug;96(8):862-71. doi: 10.1038/labinvest.2016.63. Epub 2016 Jun 13.

32.

High-throughput screening for modulators of ACVR1 transcription: discovery of potential therapeutics for fibrodysplasia ossificans progressiva.

Cappato S, Tonachini L, Giacopelli F, Tirone M, Galietta LJ, Sormani M, Giovenzana A, Spinelli AE, Canciani B, Brunelli S, Ravazzolo R, Bocciardi R.

Dis Model Mech. 2016 Jun 1;9(6):685-96. doi: 10.1242/dmm.023929. Epub 2016 Apr 28. Erratum in: Dis Model Mech. 2016 Sep 1;9(9):1067.

33.

Pharmacological analysis of epithelial chloride secretion mechanisms in adult murine airways.

Gianotti A, Ferrera L, Philp AR, Caci E, Zegarra-Moran O, Galietta LJ, Flores CA.

Eur J Pharmacol. 2016 Jun 15;781:100-8. doi: 10.1016/j.ejphar.2016.04.007. Epub 2016 Apr 7.

PMID:
27063443
34.

Evaluation of a systems biology approach to identify pharmacological correctors of the mutant CFTR chloride channel.

Pesce E, Gorrieri G, Sirci F, Napolitano F, Carrella D, Caci E, Tomati V, Zegarra-Moran O, di Bernardo D, Galietta LJ.

J Cyst Fibros. 2016 Jul;15(4):425-35. doi: 10.1016/j.jcf.2016.02.009. Epub 2016 Mar 10.

35.

The extracellular calcium-sensing receptor regulates human fetal lung development via CFTR.

Brennan SC, Wilkinson WJ, Tseng HE, Finney B, Monk B, Dibble H, Quilliam S, Warburton D, Galietta LJ, Kemp PJ, Riccardi D.

Sci Rep. 2016 Feb 25;6:21975. doi: 10.1038/srep21975.

36.

Phenylhydrazones as Correctors of a Mutant Cystic Fibrosis Transmembrane Conductance Regulator.

Nieddu E, Pollarolo B, Mazzei MT, Anzaldi M, Schenone S, Pedemonte N, Galietta LJ, Mazzei M.

Arch Pharm (Weinheim). 2016 Feb;349(2):112-23. doi: 10.1002/ardp.201500352. Epub 2015 Dec 23.

PMID:
26701662
37.

Pharmacological rescue of mutant CFTR protein improves the viscoelastic properties of CF mucus.

Gianotti A, Capurro V, Scudieri P, Galietta LJ, Moran O, Zegarra-Moran O.

J Cyst Fibros. 2016 May;15(3):295-301. doi: 10.1016/j.jcf.2015.11.003. Epub 2015 Dec 9.

38.

Novel Hits in the Correction of ΔF508-Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Protein: Synthesis, Pharmacological, and ADME Evaluation of Tetrahydropyrido[4,3-d]pyrimidines for the Potential Treatment of Cystic Fibrosis.

Pesci E, Bettinetti L, Fanti P, Galietta LJ, La Rosa S, Magnoni L, Pedemonte N, Sardone GL, Maccari L.

J Med Chem. 2015 Dec 24;58(24):9697-711. doi: 10.1021/acs.jmedchem.5b00771. Epub 2015 Dec 7.

PMID:
26561003
39.

Finding new drugs to enhance anion secretion in cystic fibrosis: Toward suitable systems for better drug screening. Report on the pre-conference meeting to the 12th ECFS Basic Science Conference, Albufeira, 25-28 March 2015.

Verkman AS, Edelman A, Amaral M, Mall MA, Beekman JM, Meiners T, Galietta LJ, Bear CE.

J Cyst Fibros. 2015 Nov;14(6):700-5. doi: 10.1016/j.jcf.2015.10.001. Epub 2015 Oct 21. No abstract available.

40.

Role of ANO4 in regulation of aldosterone secretion in the zona glomerulosa of the human adrenal gland.

Maniero C, Zhou J, Shaikh LH, Azizan EA, McFarlane I, Neogi S, Scudieri P, Galietta LJ, Brown MJ.

Lancet. 2015 Feb 26;385 Suppl 1:S62. doi: 10.1016/S0140-6736(15)60377-4.

PMID:
26312884
41.

Genetic Inhibition Of The Ubiquitin Ligase Rnf5 Attenuates Phenotypes Associated To F508del Cystic Fibrosis Mutation.

Tomati V, Sondo E, Armirotti A, Caci E, Pesce E, Marini M, Gianotti A, Jeon YJ, Cilli M, Pistorio A, Mastracci L, Ravazzolo R, Scholte B, Ronai Z, Galietta LJ, Pedemonte N.

Sci Rep. 2015 Jul 17;5:12138. doi: 10.1038/srep12138.

42.

Upregulation of TMEM16A Protein in Bronchial Epithelial Cells by Bacterial Pyocyanin.

Caci E, Scudieri P, Di Carlo E, Morelli P, Bruno S, De Fino I, Bragonzi A, Gianotti A, Sondo E, Ferrera L, Palleschi A, Santambrogio L, Ravazzolo R, Galietta LJ.

PLoS One. 2015 Jun 29;10(6):e0131775. doi: 10.1371/journal.pone.0131775. eCollection 2015.

43.

Targeting ion channels in cystic fibrosis.

Mall MA, Galietta LJ.

J Cyst Fibros. 2015 Sep;14(5):561-70. doi: 10.1016/j.jcf.2015.06.002. Epub 2015 Jun 23. Review.

44.

Ion channel and lipid scramblase activity associated with expression of TMEM16F/ANO6 isoforms.

Scudieri P, Caci E, Venturini A, Sondo E, Pianigiani G, Marchetti C, Ravazzolo R, Pagani F, Galietta LJ.

J Physiol. 2015 Sep 1;593(17):3829-48. doi: 10.1113/JP270691. Epub 2015 Jul 27.

45.

Development of the Olfactory Epithelium and Nasal Glands in TMEM16A-/- and TMEM16A+/+ Mice.

Maurya DK, Henriques T, Marini M, Pedemonte N, Galietta LJ, Rock JR, Harfe BD, Menini A.

PLoS One. 2015 Jun 11;10(6):e0129171. doi: 10.1371/journal.pone.0129171. eCollection 2015.

46.

Synthesis and structure-activity relationship of aminoarylthiazole derivatives as correctors of the chloride transport defect in cystic fibrosis.

Pesce E, Bellotti M, Liessi N, Guariento S, Damonte G, Cichero E, Galatini A, Salis A, Gianotti A, Pedemonte N, Zegarra-Moran O, Fossa P, Galietta LJ, Millo E.

Eur J Med Chem. 2015 Jun 24;99:14-35. doi: 10.1016/j.ejmech.2015.05.030. Epub 2015 May 28.

PMID:
26041577
47.

The search for a common structural moiety among selected pharmacological correctors of the mutant CFTR chloride channel.

Nieddu E, Pollarolo B, Mazzei MT, Anzaldi M, Schenone S, Pedemonte N, Pesce E, Galietta LJ, Mazzei M.

Future Med Chem. 2014;6(17):1857-68. doi: 10.4155/fmc.14.118.

PMID:
25495980
48.

Identification and characterization of a novel promoter for the human ANO1 gene regulated by the transcription factor signal transducer and activator of transcription 6 (STAT6).

Mazzone A, Gibbons SJ, Bernard CE, Nowsheen S, Middha S, Almada LL, Ordog T, Kendrick ML, Reid Lombardo KM, Shen KR, Galietta LJ, Fernandez-Zapico ME, Farrugia G.

FASEB J. 2015 Jan;29(1):152-63. doi: 10.1096/fj.14-258541. Epub 2014 Oct 28.

49.

Functional analysis of acid-activated Cl⁻ channels: properties and mechanisms of regulation.

Capurro V, Gianotti A, Caci E, Ravazzolo R, Galietta LJ, Zegarra-Moran O.

Biochim Biophys Acta. 2015 Jan;1848(1 Pt A):105-14.

50.

Anoctamin-1/TMEM16A is the major apical iodide channel of the thyrocyte.

Twyffels L, Strickaert A, Virreira M, Massart C, Van Sande J, Wauquier C, Beauwens R, Dumont JE, Galietta LJ, Boom A, Kruys V.

Am J Physiol Cell Physiol. 2014 Dec 15;307(12):C1102-12. doi: 10.1152/ajpcell.00126.2014. Epub 2014 Oct 8.

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