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Items: 1 to 50 of 249

1.

Restoration of F508-del Function by Transcomplementation: The Partners Meet in the Endoplasmic Reticulum.

Bergbower EAS, Sabirzhanova I, Boinot C, Guggino WB, Cebotaru L.

Cell Physiol Biochem. 2019;52(6):1267-1279. doi: 10.33594/000000089.

2.

Syntaxin 8 and the Endoplasmic Reticulum Processing of ΔF508-CFTR.

Sabirzhanova I, Boinot C, Guggino WB, Cebotaru L.

Cell Physiol Biochem. 2018;51(3):1489-1499. doi: 10.1159/000495596. Epub 2018 Nov 28.

3.

Role of calcium in adult onset polycystic kidney disease.

Yanda MK, Liu Q, Cebotaru V, Guggino WB, Cebotaru L.

Cell Signal. 2019 Jan;53:140-150. doi: 10.1016/j.cellsig.2018.10.003. Epub 2018 Oct 5.

PMID:
30296477
4.

Rescue of CFTR NBD2 mutants N1303K and S1235R is influenced by the functioning of the autophagosome.

Liu Q, Sabirzhanova I, Yanda MK, Bergbower EAS, Boinot C, Guggino WB, Cebotaru L.

J Cyst Fibros. 2018 Sep;17(5):582-594. doi: 10.1016/j.jcf.2018.05.016. Epub 2018 Jun 20.

5.

Wireless control of cellular function by activation of a novel protein responsive to electromagnetic fields.

Krishnan V, Park SA, Shin SS, Alon L, Tressler CM, Stokes W, Banerjee J, Sorrell ME, Tian Y, Fridman GY, Celnik P, Pevsner J, Guggino WB, Gilad AA, Pelled G.

Sci Rep. 2018 Jun 8;8(1):8764. doi: 10.1038/s41598-018-27087-9.

6.

Histone deacetylase 6 inhibition reduces cysts by decreasing cAMP and Ca2+ in knock-out mouse models of polycystic kidney disease.

Yanda MK, Liu Q, Cebotaru V, Guggino WB, Cebotaru L.

J Biol Chem. 2017 Oct 27;292(43):17897-17908. doi: 10.1074/jbc.M117.803775. Epub 2017 Sep 8.

7.

A Preclinical Study in Rhesus Macaques for Cystic Fibrosis to Assess Gene Transfer and Transduction by AAV1 and AAV5 with a Dual-Luciferase Reporter System.

Guggino WB, Benson J, Seagrave J, Yan Z, Engelhardt J, Gao G, Conlon TJ, Cebotaru L.

Hum Gene Ther Clin Dev. 2017 Sep;28(3):145-156. doi: 10.1089/humc.2017.067. Epub 2017 Jul 19.

8.

Adeno-Associated Virus (AAV) gene therapy for cystic fibrosis: current barriers and recent developments.

Guggino WB, Cebotaru L.

Expert Opin Biol Ther. 2017 Oct;17(10):1265-1273. doi: 10.1080/14712598.2017.1347630. Epub 2017 Jul 6. Review.

9.

CFTR Controls the Activity of NF-κB by Enhancing the Degradation of TRADD.

Wang H, Cebotaru L, Lee HW, Yang Q, Pollard BS, Pollard HB, Guggino WB.

Cell Physiol Biochem. 2016;40(5):1063-1078. Epub 2016 Dec 14.

10.

STIM1fl/fl Ksp-Cre Mouse has Impaired Renal Water Balance.

Cebotaru L, Cebotaru V, Wang H, Arend LJ, Guggino WB.

Cell Physiol Biochem. 2016;39(1):172-82. doi: 10.1159/000445614. Epub 2016 Jun 24.

11.

Inhibition of histone deacetylase 6 activity reduces cyst growth in polycystic kidney disease.

Cebotaru L, Liu Q, Yanda MK, Boinot C, Outeda P, Huso DL, Watnick T, Guggino WB, Cebotaru V.

Kidney Int. 2016 Jul;90(1):90-9. doi: 10.1016/j.kint.2016.01.026. Epub 2016 Mar 25.

12.

Correctors Rescue CFTR Mutations in Nucleotide-Binding Domain 1 (NBD1) by Modulating Proteostasis.

Lopes-Pacheco M, Sabirzhanova I, Rapino D, Morales MM, Guggino WB, Cebotaru L.

Chembiochem. 2016 Mar 15;17(6):493-505. doi: 10.1002/cbic.201500620. Epub 2016 Feb 16.

13.

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.

14.

Regulation of CFTR Expression and Arginine Vasopressin Activity Are Dependent on Polycystin-1 in Kidney-Derived Cells.

de Lemos Barbosa CM, Souza-Menezes J, Amaral AG, Onuchic LF, Cebotaru L, Guggino WB, Morales MM.

Cell Physiol Biochem. 2016;38(1):28-39. doi: 10.1159/000438606. Epub 2016 Jan 8.

15.

Combination of Correctors Rescue ΔF508-CFTR by Reducing Its Association with Hsp40 and Hsp27.

Lopes-Pacheco M, Boinot C, Sabirzhanova I, Morales MM, Guggino WB, Cebotaru L.

J Biol Chem. 2015 Oct 16;290(42):25636-45. doi: 10.1074/jbc.M115.671925. Epub 2015 Sep 2.

16.

Rescuing Trafficking Mutants of the ATP-binding Cassette Protein, ABCA4, with Small Molecule Correctors as a Treatment for Stargardt Eye Disease.

Sabirzhanova I, Lopes Pacheco M, Rapino D, Grover R, Handa JT, Guggino WB, Cebotaru L.

J Biol Chem. 2015 Aug 7;290(32):19743-55. doi: 10.1074/jbc.M115.647685. Epub 2015 Jun 19.

17.

Rescue of NBD2 mutants N1303K and S1235R of CFTR by small-molecule correctors and transcomplementation.

Rapino D, Sabirzhanova I, Lopes-Pacheco M, Grover R, Guggino WB, Cebotaru L.

PLoS One. 2015 Mar 23;10(3):e0119796. doi: 10.1371/journal.pone.0119796. eCollection 2015.

18.
19.

Overcoming the cystic fibrosis sputum barrier to leading adeno-associated virus gene therapy vectors.

Schuster BS, Kim AJ, Kays JC, Kanzawa MM, Guggino WB, Boyle MP, Rowe SM, Muzyczka N, Suk JS, Hanes J.

Mol Ther. 2014 Aug;22(8):1484-1493. doi: 10.1038/mt.2014.89. Epub 2014 May 29.

20.

Mis-regulation of mammalian target of rapamycin (mTOR) complexes induced by albuminuria in proximal tubules.

Peruchetti DB, Cheng J, Caruso-Neves C, Guggino WB.

J Biol Chem. 2014 Jun 13;289(24):16790-801. doi: 10.1074/jbc.M114.549717. Epub 2014 May 1.

21.

Polycystin-1 negatively regulates Polycystin-2 expression via the aggresome/autophagosome pathway.

Cebotaru V, Cebotaru L, Kim H, Chiaravalli M, Boletta A, Qian F, Guggino WB.

J Biol Chem. 2014 Mar 7;289(10):6404-14. doi: 10.1074/jbc.M113.501205. Epub 2014 Jan 23.

22.

Lung gene therapy with highly compacted DNA nanoparticles that overcome the mucus barrier.

Suk JS, Kim AJ, Trehan K, Schneider CS, Cebotaru L, Woodward OM, Boylan NJ, Boyle MP, Lai SK, Guggino WB, Hanes J.

J Control Release. 2014 Mar 28;178:8-17. doi: 10.1016/j.jconrel.2014.01.007. Epub 2014 Jan 14.

23.

Correcting the cystic fibrosis disease mutant, A455E CFTR.

Cebotaru L, Rapino D, Cebotaru V, Guggino WB.

PLoS One. 2014 Jan 8;9(1):e85183. doi: 10.1371/journal.pone.0085183. eCollection 2014.

24.

Insulin-like growth factor 1 (IGF-1) enhances the protein expression of CFTR.

Lee HW, Cheng J, Kovbasnjuk O, Donowitz M, Guggino WB.

PLoS One. 2013;8(3):e59992. doi: 10.1371/journal.pone.0059992. Epub 2013 Mar 28.

25.

Gout-causing Q141K mutation in ABCG2 leads to instability of the nucleotide-binding domain and can be corrected with small molecules.

Woodward OM, Tukaye DN, Cui J, Greenwell P, Constantoulakis LM, Parker BS, Rao A, Köttgen M, Maloney PC, Guggino WB.

Proc Natl Acad Sci U S A. 2013 Mar 26;110(13):5223-8. doi: 10.1073/pnas.1214530110. Epub 2013 Mar 14.

26.

Transcomplementation by a truncation mutant of cystic fibrosis transmembrane conductance regulator (CFTR) enhances ΔF508 processing through a biomolecular interaction.

Cebotaru L, Woodward O, Cebotaru V, Guggino WB.

J Biol Chem. 2013 Apr 12;288(15):10505-12. doi: 10.1074/jbc.M112.420489. Epub 2013 Mar 5.

27.

The GAP portion of Pseudomonas aeruginosa type III secreted toxin ExoS upregulates total and surface levels of wild type CFTR.

Tukaye DN, Kwon SH, Guggino WB.

Cell Physiol Biochem. 2013;31(1):153-65. doi: 10.1159/000343357. Epub 2013 Feb 1.

28.

Dexamethasone regulates CFTR expression in Calu-3 cells with the involvement of chaperones HSP70 and HSP90.

Prota LF, Cebotaru L, Cheng J, Wright J, Vij N, Morales MM, Guggino WB.

PLoS One. 2012;7(12):e47405. doi: 10.1371/journal.pone.0047405. Epub 2012 Dec 13.

29.

Future directions in early cystic fibrosis lung disease research: an NHLBI workshop report.

Ramsey BW, Banks-Schlegel S, Accurso FJ, Boucher RC, Cutting GR, Engelhardt JF, Guggino WB, Karp CL, Knowles MR, Kolls JK, LiPuma JJ, Lynch S, McCray PB Jr, Rubenstein RC, Singh PK, Sorscher E, Welsh M.

Am J Respir Crit Care Med. 2012 Apr 15;185(8):887-92. doi: 10.1164/rccm.201111-2068WS. Epub 2012 Feb 3.

30.

(Na+ + K+)-ATPase is a target for phosphoinositide 3-kinase/protein kinase B and protein kinase C pathways triggered by albumin.

Peruchetti DB, Pinheiro AA, Landgraf SS, Wengert M, Takiya CM, Guggino WB, Caruso-Neves C.

J Biol Chem. 2011 Dec 30;286(52):45041-7. doi: 10.1074/jbc.M111.260737. Epub 2011 Nov 4.

31.

A novel role of protein tyrosine kinase2 in mediating chloride secretion in human airway epithelial cells.

Liang L, Woodward OM, Chen Z, Cotter R, Guggino WB.

PLoS One. 2011;6(7):e21991. doi: 10.1371/journal.pone.0021991. Epub 2011 Jul 13.

32.

Polycystin-1, 2, and STIM1 interact with IP(3)R to modulate ER Ca release through the PI3K/Akt pathway.

Santoso NG, Cebotaru L, Guggino WB.

Cell Physiol Biochem. 2011;27(6):715-26. doi: 10.1159/000330080. Epub 2011 Jun 17.

33.

Methods to monitor cell surface expression and endocytic trafficking of CFTR in polarized epithelial cells.

Bomberger JM, Guggino WB, Stanton BA.

Methods Mol Biol. 2011;741:271-83. doi: 10.1007/978-1-61779-117-8_18.

34.

CFTR Folding Consortium: methods available for studies of CFTR folding and correction.

Peters KW, Okiyoneda T, Balch WE, Braakman I, Brodsky JL, Guggino WB, Penland CM, Pollard HB, Sorscher EJ, Skach WR, Thomas PJ, Lukacs GL, Frizzell RA.

Methods Mol Biol. 2011;742:335-53. doi: 10.1007/978-1-61779-120-8_20.

35.

The hypertonic environment differentially regulates wild-type CFTR and TNR-CFTR chloride channels.

Lassance-Soares RM, Cheng J, Krasnov K, Cebotaru L, Cutting GR, Souza-Menezes J, Morales MM, Guggino WB.

Cell Physiol Biochem. 2010;26(4-5):577-86. doi: 10.1159/000322325. Epub 2010 Oct 29.

36.

Identification of a polycystin-1 cleavage product, P100, that regulates store operated Ca entry through interactions with STIM1.

Woodward OM, Li Y, Yu S, Greenwell P, Wodarczyk C, Boletta A, Guggino WB, Qian F.

PLoS One. 2010 Aug 23;5(8):e12305. doi: 10.1371/journal.pone.0012305.

37.

Drosophila TRPA1 channel is required to avoid the naturally occurring insect repellent citronellal.

Kwon Y, Kim SH, Ronderos DS, Lee Y, Akitake B, Woodward OM, Guggino WB, Smith DP, Montell C.

Curr Biol. 2010 Sep 28;20(18):1672-8. doi: 10.1016/j.cub.2010.08.016.

38.

Cadmium impairs albumin reabsorption by down-regulating megalin and ClC5 channels in renal proximal tubule cells.

Gena P, Calamita G, Guggino WB.

Environ Health Perspect. 2010 Nov;118(11):1551-6. doi: 10.1289/ehp.0901874.

39.

Drosophila TRPA1 channel mediates chemical avoidance in gustatory receptor neurons.

Kim SH, Lee Y, Akitake B, Woodward OM, Guggino WB, Montell C.

Proc Natl Acad Sci U S A. 2010 May 4;107(18):8440-5. doi: 10.1073/pnas.1001425107. Epub 2010 Apr 19.

40.

Structures of a minimal human CFTR first nucleotide-binding domain as a monomer, head-to-tail homodimer, and pathogenic mutant.

Atwell S, Brouillette CG, Conners K, Emtage S, Gheyi T, Guggino WB, Hendle J, Hunt JF, Lewis HA, Lu F, Protasevich II, Rodgers LA, Romero R, Wasserman SR, Weber PC, Wetmore D, Zhang FF, Zhao X.

Protein Eng Des Sel. 2010 May;23(5):375-84. doi: 10.1093/protein/gzq004. Epub 2010 Feb 11.

PMID:
20150177
41.

Syntaxin 6 and CAL mediate the degradation of the cystic fibrosis transmembrane conductance regulator.

Cheng J, Cebotaru V, Cebotaru L, Guggino WB.

Mol Biol Cell. 2010 Apr 1;21(7):1178-87. doi: 10.1091/mbc.E09-03-0229. Epub 2010 Feb 3.

42.

Epac1 mediates protein kinase A-independent mechanism of forskolin-activated intestinal chloride secretion.

Hoque KM, Woodward OM, van Rossum DB, Zachos NC, Chen L, Leung GP, Guggino WB, Guggino SE, Tse CM.

J Gen Physiol. 2010 Jan;135(1):43-58. doi: 10.1085/jgp.200910339.

43.

Structure and dynamics of NBD1 from CFTR characterized using crystallography and hydrogen/deuterium exchange mass spectrometry.

Lewis HA, Wang C, Zhao X, Hamuro Y, Conners K, Kearins MC, Lu F, Sauder JM, Molnar KS, Coales SJ, Maloney PC, Guggino WB, Wetmore DR, Weber PC, Hunt JF.

J Mol Biol. 2010 Feb 19;396(2):406-30. doi: 10.1016/j.jmb.2009.11.051. Epub 2009 Nov 26.

PMID:
19944699
44.

WNK4 enhances the degradation of NCC through a sortilin-mediated lysosomal pathway.

Zhou B, Zhuang J, Gu D, Wang H, Cebotaru L, Guggino WB, Cai H.

J Am Soc Nephrol. 2010 Jan;21(1):82-92. doi: 10.1681/ASN.2008121275. Epub 2009 Oct 29.

45.

Polycystin-1 interacts with inositol 1,4,5-trisphosphate receptor to modulate intracellular Ca2+ signaling with implications for polycystic kidney disease.

Li Y, Santoso NG, Yu S, Woodward OM, Qian F, Guggino WB.

J Biol Chem. 2009 Dec 25;284(52):36431-41. doi: 10.1074/jbc.M109.068916. Epub 2009 Oct 23.

46.

Dual reporter comparative indexing of rAAV pseudotyped vectors in chimpanzee airway.

Flotte TR, Fischer AC, Goetzmann J, Mueller C, Cebotaru L, Yan Z, Wang L, Wilson JM, Guggino WB, Engelhardt JF.

Mol Ther. 2010 Mar;18(3):594-600. doi: 10.1038/mt.2009.230. Epub 2009 Oct 13.

47.

Identification of a urate transporter, ABCG2, with a common functional polymorphism causing gout.

Woodward OM, Köttgen A, Coresh J, Boerwinkle E, Guggino WB, Köttgen M.

Proc Natl Acad Sci U S A. 2009 Jun 23;106(25):10338-42. doi: 10.1073/pnas.0901249106. Epub 2009 Jun 8.

48.

Hypoxia-induced mitogenic factor/FIZZ1 induces intracellular calcium release through the PLC-IP(3) pathway.

Fan C, Su Q, Li Y, Liang L, Angelini DJ, Guggino WB, Johns RA.

Am J Physiol Lung Cell Mol Physiol. 2009 Aug;297(2):L263-70. doi: 10.1152/ajplung.90416.2008. Epub 2009 May 8.

49.

Spiperone, identified through compound screening, activates calcium-dependent chloride secretion in the airway.

Liang L, MacDonald K, Schwiebert EM, Zeitlin PL, Guggino WB.

Am J Physiol Cell Physiol. 2009 Jan;296(1):C131-41. doi: 10.1152/ajpcell.00346.2008. Epub 2008 Nov 5.

50.

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