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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.


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.


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.


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.


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.


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.


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.


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.


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.


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.


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.


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.


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.


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.


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.


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.


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.


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.


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.

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