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Items: 1 to 50 of 133

1.

High-throughput phenotyping of heteromeric human ether-à-go-go-related gene potassium channel variants can discriminate pathogenic from rare benign variants.

Ng CA, Perry MD, Liang W, Smith NJ, Foo B, Shrier A, Lukacs GL, Hill AP, Vandenberg JI.

Heart Rhythm. 2019 Sep 23. pii: S1547-5271(19)30853-7. doi: 10.1016/j.hrthm.2019.09.020. [Epub ahead of print]

PMID:
31557540
2.

Phosphorylation-dependent modulation of CFTR macromolecular signalling complex activity by cigarette smoke condensate in airway epithelia.

Schnúr A, Premchandar A, Bagdany M, Lukacs GL.

Sci Rep. 2019 Sep 3;9(1):12706. doi: 10.1038/s41598-019-48971-y.

3.

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.

4.

Single Cell Fluorescence Ratio Image Analysis for Studying ESCRT Function in Receptor Trafficking.

Kazan JM, Lukacs GL, Apaja PM, Pause A.

Methods Mol Biol. 2019;1998:93-103. doi: 10.1007/978-1-4939-9492-2_7.

PMID:
31250296
5.

Mutation-specific peripheral and ER quality control of hERG channel cell-surface expression.

Foo B, Barbier C, Guo K, Vasantharuban J, Lukacs GL, Shrier A.

Sci Rep. 2019 Apr 15;9(1):6066. doi: 10.1038/s41598-019-42331-6.

6.

Transcytosis maintains CFTR apical polarity in the face of constitutive and mutation-induced basolateral missorting.

Bidaud-Meynard A, Bossard F, Schnúr A, Fukuda R, Veit G, Xu H, Lukacs GL.

J Cell Sci. 2019 May 15;132(10). pii: jcs226886. doi: 10.1242/jcs.226886.

PMID:
30975917
7.

Identification of Allosteric Inhibitors against Active Caspase-6.

Tubeleviciute-Aydin A, Beautrait A, Lynham J, Sharma G, Gorelik A, Deny LJ, Soya N, Lukacs GL, Nagar B, Marinier A, LeBlanc AC.

Sci Rep. 2019 Apr 2;9(1):5504. doi: 10.1038/s41598-019-41930-7.

8.

Differential Scanning Fluorimetry and Hydrogen Deuterium Exchange Mass Spectrometry to Monitor the Conformational Dynamics of NBD1 in Cystic Fibrosis.

Soya N, Roldan A, Lukacs GL.

Methods Mol Biol. 2019;1873:53-67. doi: 10.1007/978-1-4939-8820-4_4.

PMID:
30341603
9.

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

Veit G, Xu H, Dreano E, Avramescu RG, Bagdany M, Beitel LK, Roldan A, Hancock MA, Lay C, Li W, Morin K, Gao S, Mak PA, Ainscow E, Orth AP, McNamara P, Edelman A, Frenkiel S, Matouk E, Sermet-Gaudelus I, Barnes WG, Lukacs GL.

Nat Med. 2018 Nov;24(11):1732-1742. doi: 10.1038/s41591-018-0200-x. Epub 2018 Oct 8.

10.

Publisher Correction: Mechanism of parkin activation by phosphorylation.

Sauvé V, Sung G, Soya N, Kozlov G, Blaimschein N, Miotto LS, Trempe JF, Lukacs GL, Gehring K.

Nat Struct Mol Biol. 2018 Aug;25(8):744. doi: 10.1038/s41594-018-0105-x.

PMID:
30026521
11.

Mechanism of parkin activation by phosphorylation.

Sauvé V, Sung G, Soya N, Kozlov G, Blaimschein N, Miotto LS, Trempe JF, Lukacs GL, Gehring K.

Nat Struct Mol Biol. 2018 Jul;25(7):623-630. doi: 10.1038/s41594-018-0088-7. Epub 2018 Jul 2. Erratum in: Nat Struct Mol Biol. 2018 Aug;25(8):744.

PMID:
29967542
12.

ΔF508-CFTR Modulator Screen Based on Cell Surface Targeting of a Chimeric Nucleotide Binding Domain 1 Reporter.

Phuan PW, Veit G, Tan JA, Roldan A, Finkbeiner WE, Haggie PM, Lukacs GL, Verkman AS.

SLAS Discov. 2018 Sep;23(8):823-831. doi: 10.1177/2472555218763310. Epub 2018 Mar 13.

13.

Chaperone-Independent Peripheral Quality Control of CFTR by RFFL E3 Ligase.

Okiyoneda T, Veit G, Sakai R, Aki M, Fujihara T, Higashi M, Susuki-Miyata S, Miyata M, Fukuda N, Yoshida A, Xu H, Apaja PM, Lukacs GL.

Dev Cell. 2018 Mar 26;44(6):694-708.e7. doi: 10.1016/j.devcel.2018.02.001. Epub 2018 Mar 1.

14.

PINK1 autophosphorylation is required for ubiquitin recognition.

Rasool S, Soya N, Truong L, Croteau N, Lukacs GL, Trempe JF.

EMBO Rep. 2018 Apr;19(4). pii: e44981. doi: 10.15252/embr.201744981. Epub 2018 Feb 23.

15.

Extracellular oxidation in cystic fibrosis airway epithelium causes enhanced EGFR/ADAM17 activity.

Stolarczyk M, Veit G, Schnúr A, Veltman M, Lukacs GL, Scholte BJ.

Am J Physiol Lung Cell Mol Physiol. 2018 Apr 1;314(4):L555-L568. doi: 10.1152/ajplung.00458.2017. Epub 2017 Dec 14.

16.

Hormonal vitamin D up-regulates tissue-specific PD-L1 and PD-L2 surface glycoprotein expression in humans but not mice.

Dimitrov V, Bouttier M, Boukhaled G, Salehi-Tabar R, Avramescu RG, Memari B, Hasaj B, Lukacs GL, Krawczyk CM, White JH.

J Biol Chem. 2017 Dec 15;292(50):20657-20668. doi: 10.1074/jbc.M117.793885. Epub 2017 Oct 23.

17.

Mutation-specific downregulation of CFTR2 variants by gating potentiators.

Avramescu RG, Kai Y, Xu H, Bidaud-Meynard A, Schnúr A, Frenkiel S, Matouk E, Veit G, Lukacs GL.

Hum Mol Genet. 2017 Dec 15;26(24):4873-4885. doi: 10.1093/hmg/ddx367.

18.

Leukoencephalopathy-causing CLCN2 mutations are associated with impaired Cl- channel function and trafficking.

Gaitán-Peñas H, Apaja PM, Arnedo T, Castellanos A, Elorza-Vidal X, Soto D, Gasull X, Lukacs GL, Estévez R.

J Physiol. 2017 Nov 15;595(22):6993-7008. doi: 10.1113/JP275087. Epub 2017 Oct 9.

19.

Chaperones rescue the energetic landscape of mutant CFTR at single molecule and in cell.

Bagdany M, Veit G, Fukuda R, Avramescu RG, Okiyoneda T, Baaklini I, Singh J, Sovak G, Xu H, Apaja PM, Sattin S, Beitel LK, Roldan A, Colombo G, Balch W, Young JC, Lukacs GL.

Nat Commun. 2017 Aug 30;8(1):398. doi: 10.1038/s41467-017-00444-4.

20.

Single-particle electron microscopy structure of UDP-glucose:glycoprotein glucosyltransferase suggests a selectivity mechanism for misfolded proteins.

Calles-Garcia D, Yang M, Soya N, Melero R, Ménade M, Ito Y, Vargas J, Lukacs GL, Kollman JM, Kozlov G, Gehring K.

J Biol Chem. 2017 Jul 7;292(27):11499-11507. doi: 10.1074/jbc.M117.789495. Epub 2017 May 10.

21.

Correctors and Potentiators Rescue Function of the Truncated W1282X-Cystic Fibrosis Transmembrane Regulator (CFTR) Translation Product.

Haggie PM, Phuan PW, Tan JA, Xu H, Avramescu RG, Perdomo D, Zlock L, Nielson DW, Finkbeiner WE, Lukacs GL, Verkman AS.

J Biol Chem. 2017 Jan 20;292(3):771-785. doi: 10.1074/jbc.M116.764720. Epub 2016 Nov 28.

22.

New insights into interactions between the nucleotide-binding domain of CFTR and keratin 8.

Premchandar A, Kupniewska A, Bonna A, Faure G, Fraczyk T, Roldan A, Hoffmann B, Faria da Cunha M, Herrmann H, Lukacs GL, Edelman A, Dadlez M.

Protein Sci. 2017 Feb;26(2):343-354. doi: 10.1002/pro.3086.

23.

A Christianson syndrome-linked deletion mutation (∆(287)ES(288)) in SLC9A6 disrupts recycling endosomal function and elicits neurodegeneration and cell death.

Ilie A, Gao AY, Reid J, Boucher A, McEwan C, Barrière H, Lukacs GL, McKinney RA, Orlowski J.

Mol Neurodegener. 2016 Sep 2;11(1):63. doi: 10.1186/s13024-016-0129-9.

24.

Epithelial Anion Transport as Modulator of Chemokine Signaling.

Schnúr A, Hegyi P, Rousseau S, Lukacs GL, Veit G.

Mediators Inflamm. 2016;2016:7596531. doi: 10.1155/2016/7596531. Epub 2016 Jun 12. Review.

25.

Rattlesnake Phospholipase A2 Increases CFTR-Chloride Channel Current and Corrects ∆F508CFTR Dysfunction: Impact in Cystic Fibrosis.

Faure G, Bakouh N, Lourdel S, Odolczyk N, Premchandar A, Servel N, Hatton A, Ostrowski MK, Xu H, Saul FA, Moquereau C, Bitam S, Pranke I, Planelles G, Teulon J, Herrmann H, Roldan A, Zielenkiewicz P, Dadlez M, Lukacs GL, Sermet-Gaudelus I, Ollero M, Corringer PJ, Edelman A.

J Mol Biol. 2016 Jul 17;428(14):2898-915. doi: 10.1016/j.jmb.2016.05.016. Epub 2016 May 27.

PMID:
27241308
26.

Proteostasis: Chaperoning for hearing loss.

Lukacs GL.

Nat Chem Biol. 2016 May 18;12(6):388-9. doi: 10.1038/nchembio.2091. No abstract available.

PMID:
27191645
27.

Development and characterization of synthetic antibodies binding to the cystic fibrosis conductance regulator.

Gakhal AK, Jensen TJ, Bozoky Z, Roldan A, Lukacs GL, Forman-Kay J, Riordan JR, Sidhu SS.

MAbs. 2016 Aug-Sep;8(6):1167-76. doi: 10.1080/19420862.2016.1186320. Epub 2016 May 16.

28.

Ribosomal Stalk Protein Silencing Partially Corrects the ΔF508-CFTR Functional Expression Defect.

Veit G, Oliver K, Apaja PM, Perdomo D, Bidaud-Meynard A, Lin ST, Guo J, Icyuz M, Sorscher EJ, Hartman JL IV, Lukacs GL.

PLoS Biol. 2016 May 11;14(5):e1002462. doi: 10.1371/journal.pbio.1002462. eCollection 2016 May. Erratum in: PLoS Biol. 2016 Nov 2;14 (11):e1002574.

29.

CFTR: A New Horizon in the Pathomechanism and Treatment of Pancreatitis.

Hegyi P, Wilschanski M, Muallem S, Lukacs GL, Sahin-Tóth M, Uc A, Gray MA, Rakonczay Z Jr, Maléth J.

Rev Physiol Biochem Pharmacol. 2016;170:37-66. doi: 10.1007/112_2015_5002. Review.

30.

From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations.

Veit G, Avramescu RG, Chiang AN, Houck SA, Cai Z, Peters KW, Hong JS, Pollard HB, Guggino WB, Balch WE, Skach WR, Cutting GR, Frizzell RA, Sheppard DN, Cyr DM, Sorscher EJ, Brodsky JL, Lukacs GL.

Mol Biol Cell. 2016 Feb 1;27(3):424-33. doi: 10.1091/mbc.E14-04-0935.

31.

Non-native Conformers of Cystic Fibrosis Transmembrane Conductance Regulator NBD1 Are Recognized by Hsp27 and Conjugated to SUMO-2 for Degradation.

Gong X, Ahner A, Roldan A, Lukacs GL, Thibodeau PH, Frizzell RA.

J Biol Chem. 2016 Jan 22;291(4):2004-17. doi: 10.1074/jbc.M115.685628. Epub 2015 Dec 1.

32.

Channel Gating Regulation by the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) First Cytosolic Loop.

Ehrhardt A, Chung WJ, Pyle LC, Wang W, Nowotarski K, Mulvihill CM, Ramjeesingh M, Hong J, Velu SE, Lewis HA, Atwell S, Aller S, Bear CE, Lukacs GL, Kirk KL, Sorscher EJ.

J Biol Chem. 2016 Jan 22;291(4):1854-65. doi: 10.1074/jbc.M115.704809. Epub 2015 Dec 1.

33.

Interplay of Endosomal pH and Ligand Occupancy in Integrin α5β1 Ubiquitination, Endocytic Sorting, and Cell Migration.

Kharitidi D, Apaja PM, Manteghi S, Suzuki K, Malitskaya E, Roldan A, Gingras MC, Takagi J, Lukacs GL, Pause A.

Cell Rep. 2015 Oct 20;13(3):599-609. doi: 10.1016/j.celrep.2015.09.024. Epub 2015 Oct 8.

34.

Potentiators of Defective ΔF508-CFTR Gating that Do Not Interfere with Corrector Action.

Phuan PW, Veit G, Tan JA, Finkbeiner WE, Lukacs GL, Verkman AS.

Mol Pharmacol. 2015 Oct;88(4):791-9. doi: 10.1124/mol.115.099689. Epub 2015 Aug 5.

35.

Alcohol disrupts levels and function of the cystic fibrosis transmembrane conductance regulator to promote development of pancreatitis.

Maléth J, Balázs A, Pallagi P, Balla Z, Kui B, Katona M, Judák L, Németh I, Kemény LV, Rakonczay Z Jr, Venglovecz V, Földesi I, Pető Z, Somorácz Á, Borka K, Perdomo D, Lukacs GL, Gray MA, Monterisi S, Zaccolo M, Sendler M, Mayerle J, Kühn JP, Lerch MM, Sahin-Tóth M, Hegyi P.

Gastroenterology. 2015 Feb;148(2):427-39.e16. doi: 10.1053/j.gastro.2014.11.002. Epub 2014 Nov 7.

36.

Some gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression.

Veit G, Avramescu RG, Perdomo D, Phuan PW, Bagdany M, Apaja PM, Borot F, Szollosi D, Wu YS, Finkbeiner WE, Hegedus T, Verkman AS, Lukacs GL.

Sci Transl Med. 2014 Jul 23;6(246):246ra97. doi: 10.1126/scitranslmed.3008889.

37.

Protein homeostasis at the plasma membrane.

Apaja PM, Lukacs GL.

Physiology (Bethesda). 2014 Jul;29(4):265-77. doi: 10.1152/physiol.00058.2013. Review.

38.

Degradation mechanism of a Golgi-retained distal renal tubular acidosis mutant of the kidney anion exchanger 1 in renal cells.

Chu CY, King J, Berrini M, Rumley AC, Apaja PM, Lukacs GL, Alexander RT, Cordat E.

Am J Physiol Cell Physiol. 2014 Aug 1;307(3):C296-307. doi: 10.1152/ajpcell.00310.2013. Epub 2014 Jun 11.

39.

Synergy-based small-molecule screen using a human lung epithelial cell line yields ΔF508-CFTR correctors that augment VX-809 maximal efficacy.

Phuan PW, Veit G, Tan J, Roldan A, Finkbeiner WE, Lukacs GL, Verkman AS.

Mol Pharmacol. 2014 Jul;86(1):42-51. doi: 10.1124/mol.114.092478. Epub 2014 Apr 15.

40.

Ubiquitin-dependent sorting in endocytosis.

Piper RC, Dikic I, Lukacs GL.

Cold Spring Harb Perspect Biol. 2014 Jan 1;6(1). pii: a016808. doi: 10.1101/cshperspect.a016808. Review.

41.

Ubiquitination-dependent quality control of hERG K+ channel with acquired and inherited conformational defect at the plasma membrane.

Apaja PM, Foo B, Okiyoneda T, Valinsky WC, Barriere H, Atanasiu R, Ficker E, Lukacs GL, Shrier A.

Mol Biol Cell. 2013 Dec;24(24):3787-804. doi: 10.1091/mbc.E13-07-0417. Epub 2013 Oct 23.

42.

Discovery of novel potent ΔF508-CFTR correctors that target the nucleotide binding domain.

Odolczyk N, Fritsch J, Norez C, Servel N, da Cunha MF, Bitam S, Kupniewska A, Wiszniewski L, Colas J, Tarnowski K, Tondelier D, Roldan A, Saussereau EL, Melin-Heschel P, Wieczorek G, Lukacs GL, Dadlez M, Faure G, Herrmann H, Ollero M, Becq F, Zielenkiewicz P, Edelman A.

EMBO Mol Med. 2013 Oct;5(10):1484-501. doi: 10.1002/emmm.201302699. Epub 2013 Aug 27.

43.

Insights into MLC pathogenesis: GlialCAM is an MLC1 chaperone required for proper activation of volume-regulated anion currents.

Capdevila-Nortes X, López-Hernández T, Apaja PM, López de Heredia M, Sirisi S, Callejo G, Arnedo T, Nunes V, Lukacs GL, Gasull X, Estévez R.

Hum Mol Genet. 2013 Nov 1;22(21):4405-16. doi: 10.1093/hmg/ddt290. Epub 2013 Jun 20.

PMID:
23793458
44.

Mechanism-based corrector combination restores ΔF508-CFTR folding and function.

Okiyoneda T, Veit G, Dekkers JF, Bagdany M, Soya N, Xu H, Roldan A, Verkman AS, Kurth M, Simon A, Hegedus T, Beekman JM, Lukacs GL.

Nat Chem Biol. 2013 Jul;9(7):444-54. doi: 10.1038/nchembio.1253. Epub 2013 May 12.

45.

Small heat shock proteins target mutant cystic fibrosis transmembrane conductance regulator for degradation via a small ubiquitin-like modifier-dependent pathway.

Ahner A, Gong X, Schmidt BZ, Peters KW, Rabeh WM, Thibodeau PH, Lukacs GL, Frizzell RA.

Mol Biol Cell. 2013 Jan;24(2):74-84. doi: 10.1091/mbc.E12-09-0678. Epub 2012 Nov 14.

46.

Reduced PDZ interactions of rescued ΔF508CFTR increases its cell surface mobility.

Valentine CD, Lukacs GL, Verkman AS, Haggie PM.

J Biol Chem. 2012 Dec 21;287(52):43630-8. doi: 10.1074/jbc.M112.421172. Epub 2012 Oct 31.

47.

Fixing cystic fibrosis by correcting CFTR domain assembly.

Okiyoneda T, Lukacs GL.

J Cell Biol. 2012 Oct 15;199(2):199-204. doi: 10.1083/jcb.201208083.

48.

Proinflammatory cytokine secretion is suppressed by TMEM16A or CFTR channel activity in human cystic fibrosis bronchial epithelia.

Veit G, Bossard F, Goepp J, Verkman AS, Galietta LJ, Hanrahan JW, Lukacs GL.

Mol Biol Cell. 2012 Nov;23(21):4188-202. doi: 10.1091/mbc.E12-06-0424. Epub 2012 Sep 12.

49.

Comparative processing and function of human and ferret cystic fibrosis transmembrane conductance regulator.

Fisher JT, Liu X, Yan Z, Luo M, Zhang Y, Zhou W, Lee BJ, Song Y, Guo C, Wang Y, Lukacs GL, Engelhardt JF.

J Biol Chem. 2012 Jun 22;287(26):21673-85. doi: 10.1074/jbc.M111.336537. Epub 2012 May 8.

50.

Correction of both NBD1 energetics and domain interface is required to restore ΔF508 CFTR folding and function.

Rabeh WM, Bossard F, Xu H, Okiyoneda T, Bagdany M, Mulvihill CM, Du K, di Bernardo S, Liu Y, Konermann L, Roldan A, Lukacs GL.

Cell. 2012 Jan 20;148(1-2):150-63. doi: 10.1016/j.cell.2011.11.024.

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