Research output per year
Research output per year
- 1 - 50 out of 309 results
Search results
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2023
CFTR and PC2, partners in the primary cilia in autosomal dominant polycystic kidney disease
Yanda, M. K., Ciobanu, C., Guggino, W. B. & Cebotaru, L., Sep 1 2023, In: American Journal of Physiology. 325, 3, p. C682-C693Research output: Contribution to journal › Article › peer-review
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Transduction of Ferret Surface and Basal Cells of Airways, Lung, Liver, and Pancreas via Intratracheal or Intravenous Delivery of Adeno-Associated Virus 1 or 6
Yanda, M. K., Zeidan, A., Ciobanu, C., Izzi, J., Guggino, W. B. & Cebotaru, L., Nov 1 2023, In: Human gene therapy. 34, 21-22, p. 1135-1144 10 p.Research output: Contribution to journal › Article › peer-review
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2022
Short-Term Steroid Treatment of Rhesus Macaque Increases Transduction
Yanda, M. K., Tomar, V., Cebotaru, C. V., Guggino, W. B. & Cebotaru, L., Feb 2022, In: Human gene therapy. 33, 3-4, p. 131-147 17 p.Research output: Contribution to journal › Article › peer-review
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The Mitochondrial Ca2+ import complex is altered in ADPKD
Yanda, M. K., Tomar, V., Cole, R., Guggino, W. B. & Cebotaru, L., Jan 2022, In: Cell Calcium. 101, 102501.Research output: Contribution to journal › Article › peer-review
Open Access -
2021
Megalin-mediated albumin endocytosis in renal proximal tubules is involved in the antiproteinuric effect of angiotensin II type 1 receptor blocker in a subclinical acute kidney injury animal model
Peruchetti, D. B., Barahuna-Filho, P. F. R., Silva-Aguiar, R. P., Abreu, T. P., Takiya, C. M., Cheng, J., Pinheiro, A. A. S., Cebotaru, L., Guggino, W. B. & Caruso-Neves, C., Sep 2021, In: Biochimica et Biophysica Acta - General Subjects. 1865, 9, 129950.Research output: Contribution to journal › Article › peer-review
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2020
A new role for heat shock factor 27 in the pathophysiology of Clostridium difficile toxin B
Yanda, M. K., Guggino, W. B. & Cebotaru, L., Jan 1 2020, In: American journal of physiology. Gastrointestinal and liver physiology. 318, 1, p. G120-G129Research output: Contribution to journal › Article › peer-review
Open Access -
Gene Therapy for Cystic Fibrosis Paved the Way for the Use of Adeno-Associated Virus in Gene Therapy
Guggino, W. B. & Cebotaru, L., May 2020, In: Human gene therapy. 31, 9-10, p. 538-541 4 p.Research output: Contribution to journal › Article › peer-review
Open Access2 Scopus citations -
Transduction of Surface and Basal Cells in Rhesus Macaque Lung following Repeat Dosing with AAV1CFTR
Guggino, W. B., Yanda, M. K., Cebotaru, C. V. & Cebotaru, L., Sep 2020, In: Human gene therapy. 31, 17-18, p. 1010-1023 14 p.Research output: Contribution to journal › Article › peer-review
1 Scopus citations -
2019
Restoration of F508-del function by transcomplementation: The partners meet in the endoplasmic reticulum
Anne, E., Bergbower, S., Sabirzhanova, I., Boinot, C., Guggino, W. B. & Cebotaru, L., 2019, In: Cellular Physiology and Biochemistry. 52, 6, p. 1267-1279 13 p.Research output: Contribution to journal › Article › peer-review
Open Access1 Scopus citations -
Role of calcium in adult onset polycystic kidney disease
Yanda, M. K., Liu, Q., Cebotaru, V., Guggino, W. B. & Cebotaru, L., Jan 2019, In: Cellular Signalling. 53, p. 140-150 11 p.Research output: Contribution to journal › Article › peer-review
3 Scopus citations -
The CFTR corrector, VX-809 (Lumacaftor), rescues ABCA4 trafficking mutants: A potential treatment for stargardt disease
Liu, Q., Sabirzhanova, I., Bergbower, E. A. S., Yanda, M., Guggino, W. G. & Cebotaru, L., 2019, In: Cellular Physiology and Biochemistry. 53, 2, p. 400-412 13 p.Research output: Contribution to journal › Article › peer-review
Open Access6 Scopus citations -
2018
Rescue of CFTR NBD2 mutants N1303K and S1235R is influenced by the functioning of the autophagosome
Liu, Q., Sabirzhanova, I., Yanda, M. K., Bergbower, E. A. S., Boinot, C., Guggino, W. B. & Cebotaru, L., Sep 2018, In: Journal of Cystic Fibrosis. 17, 5, p. 582-594 13 p.Research output: Contribution to journal › Article › peer-review
Open Access5 Scopus citations -
Syntaxin 8 and the endoplasmic reticulum processing of Δf508-CFTR
Sabirzhanova, I., Boinot, C., Guggino, W. B. & Cebotaru, L., Dec 1 2018, In: Cellular Physiology and Biochemistry. 51, 3, p. 1489-1499 11 p.Research output: Contribution to journal › Article › peer-review
Open Access2 Scopus citations -
The CFTR-Associated ligand arrests the trafficking of the mutant Δf508 CFTR channel in the ER contributing to cystic fibrosis
Bergbower, E., Boinot, C., Sabirzhanova, I., Guggino, W. & Cebotaru, L., Feb 2018, In: Cellular Physiology and Biochemistry. 45, 2, p. 639-655 17 p.Research output: Contribution to journal › Article › peer-review
Open Access6 Scopus citations -
Wireless control of cellular function by activation of a novel protein responsive to electromagnetic fields
Krishnan, V., Park, S. A., Shin, S. S., Alon, L., Tressler, C. M., Stokes, W., Banerjee, J., Sorrell, M. E., Tian, Y., Fridman, G. Y., Celnik, P., Pevsner, J., Guggino, W. B., Gilad, A. A. & Pelled, G., Dec 1 2018, In: Scientific reports. 8, 1, 8764.Research output: Contribution to journal › Article › peer-review
Open Access10 Scopus citations -
2017
Adeno-Associated Virus (AAV) gene therapy for cystic fibrosis: current barriers and recent developments
Guggino, W. B. & Cebotaru, L., Oct 3 2017, In: Expert Opinion on Biological Therapy. 17, 10, p. 1265-1273 9 p.Research output: Contribution to journal › Review article › peer-review
23 Scopus citations -
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, W. B., Benson, J., Seagrave, J., Yan, Z., Engelhardt, J., Gao, G., Conlon, T. J. & Cebotaru, L., Sep 2017, In: Human gene therapy. Clinical development. 28, 3, p. 145-156 12 p.Research output: Contribution to journal › Article › peer-review
10 Scopus citations -
Histone deacetylase 6 inhibition reduces cysts by decreasing cAMP and Ca2 in knock-out mouse models of polycystic kidney disease
Yanda, M. K., Liu, Q., Cebotaru, V., Guggino, W. B. & Cebotaru, L., Oct 27 2017, In: Journal of Biological Chemistry. 292, 43, p. 17897-17908 12 p.Research output: Contribution to journal › Article › peer-review
Open Access16 Scopus citations -
2016
CFTR Controls the Activity of NF-κB by Enhancing the Degradation of TRADD
Wang, H., Cebotaru, L., Lee, H. W., Yang, Q., Pollard, B. S., Pollard, H. B. & Guggino, W. B., Dec 1 2016, In: Cellular Physiology and Biochemistry. 40, 5, p. 1063-1078 16 p.Research output: Contribution to journal › Article › peer-review
Open Access18 Scopus citations -
Correctors Rescue CFTR Mutations in Nucleotide-Binding Domain 1 (NBD1) by Modulating Proteostasis
Lopes-Pacheco, M., Sabirzhanova, I., Rapino, D., Morales, M. M., Guggino, W. B. & Cebotaru, L., Mar 15 2016, In: ChemBioChem. 17, 6, p. 493-505 13 p.Research output: Contribution to journal › Article › peer-review
13 Scopus citations -
From CFTR biology toward combinatorial pharmacotherapy: Expanded classification of cystic fibrosis mutations
Veit, G., Avramescu, R. G., Chiang, A. N., Houck, S. A., Cai, Z., Peters, K. W., Hong, J. S., Pollard, H. B., Guggino, W. B., Balch, W. E., Skach, W. R., Cutting, G. R., Frizzell, R. A., Sheppard, D. N., Cyr, D. M., Sorscher, E. J., Brodsky, J. L. & Lukacs, G. L., Feb 1 2016, In: Molecular biology of the cell. 27, 3, p. 424-433 10 p.Research output: Contribution to journal › Article › peer-review
Open Access219 Scopus citations -
Inhibition of histone deacetylase 6 activity reduces cyst growth in polycystic kidney disease
Cebotaru, L., Liu, Q., Yanda, M. K., Boinot, C., Outeda, P., Huso, D. L., Watnick, T., Guggino, W. B. & Cebotaru, V., Jul 1 2016, In: Kidney international. 90, 1, p. 90-99 10 p.Research output: Contribution to journal › Article › peer-review
Open Access41 Scopus citations -
Regulation of CFTR Expression and Arginine Vasopressin Activity Are Dependent on Polycystin-1 in Kidney-Derived Cells
De Lemos Barbosa, C. M., Souza-Menezes, J., Amaral, A. G., Onuchic, L. F., Cebotaru, L., Guggino, W. B. & Morales, M. M., Jan 1 2016, In: Cellular Physiology and Biochemistry. 38, 1, p. 28-39 12 p.Research output: Contribution to journal › Article › peer-review
Open Access6 Scopus citations -
STIM1 fl/fl Ksp-Cre Mouse has Impaired Renal Water Balance
Cebotaru, L., Cebotaru, V., Wang, H., Arend, L. J. & Guggino, W. B., Jul 1 2016, In: Cellular Physiology and Biochemistry. 39, 1, p. 172-182 11 p.Research output: Contribution to journal › Article › peer-review
Open Access2 Scopus citations -
2015
Combination of correctors rescue AF508-CFTR by reducing its association with Hsp40 and Hsp27
Lopes-Pacheco, M., Boinot, C., Sabirzhanova, I., Morales, M. M., Guggino, W. B. & Cebotaru, L., Oct 16 2015, In: Journal of Biological Chemistry. 290, 42, p. 25636-25645 10 p.Research output: Contribution to journal › Article › peer-review
Open Access22 Scopus citations -
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, W. B. & Cebotaru, L., Mar 23 2015, In: PloS one. 10, 3, e0119796.Research output: Contribution to journal › Article › peer-review
Open Access23 Scopus citations -
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, J. T., Guggino, W. B. & Cebotaru, L., Aug 7 2015, In: Journal of Biological Chemistry. 290, 32, p. 19743-19755 13 p.Research output: Contribution to journal › Article › peer-review
Open Access31 Scopus citations -
2014
Complement yourself: Transcomplementation rescues partially folded mutant proteins
Cebotaru, L. & Guggino, W. B., Mar 2014, In: Biophysical Reviews. 6, 1, p. 169-180 12 p.Research output: Contribution to journal › Review article › peer-review
Open Access4 Scopus citations -
Correcting the cystic fibrosis disease mutant, A455E CFTR
Cebotaru, L., Rapino, D., Cebotaru, V. & Guggino, W. B., Jan 8 2014, In: PloS one. 9, 1, e85183.Research output: Contribution to journal › Article › peer-review
Open Access12 Scopus citations -
Lung gene therapy with highly compacted DNA nanoparticles that overcome the mucus barrier
Suk, J. S., Kim, A. J., Trehan, K., Schneider, C. S., Cebotaru, L., Woodward, O. M., Boylan, N. J., Boyle, M. P., Lai, S. K., Guggino, W. B. & Hanes, J., Mar 28 2014, In: Journal of Controlled Release. 178, 1, p. 8-17 10 p.Research output: Contribution to journal › Article › peer-review
Open Access121 Scopus citations -
Mis-regulation of mammalian target of rapamycin (mTOR) complexes induced by albuminuria in proximal tubules
Peruchetti, D. B., Cheng, J., Caruso-Neves, C. & Guggino, W. B., Jun 13 2014, In: Journal of Biological Chemistry. 289, 24, p. 16790-16801 12 p.Research output: Contribution to journal › Article › peer-review
Open Access21 Scopus citations -
Overcoming the cystic fibrosis sputum barrier to leading adeno-associated virus gene therapy vectors
Schuster, B. S., Kim, A. J., Kays, J. C., Kanzawa, M. M., Guggino, W. B., Boyle, M. P., Rowe, S. M., Muzyczka, N., Suk, J. S. & Hanes, J., Aug 2014, In: Molecular Therapy. 22, 8, p. 1484-1493 10 p.Research output: Contribution to journal › Article › peer-review
Open Access53 Scopus citations -
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, W. B., Mar 7 2014, In: Journal of Biological Chemistry. 289, 10, p. 6404-6414 11 p.Research output: Contribution to journal › Article › peer-review
Open Access19 Scopus citations -
2013
Anion Channels
Woodward, O. M. & Guggino, W. B., Aug 29 2013, Seldin and Geibisch's The Kidney. Elsevier Inc., Vol. 1. p. 1019-1045 27 p.Research output: Chapter in Book/Report/Conference proceeding › Chapter
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Generation and genetic engineering of human induced pluripotent stem cells using designed zinc finger nucleases
Ramalingam, S., London, V., Kandavelou, K., Cebotaru, L., Guggino, W., Civin, C. & Chandrasegaran, S., Feb 15 2013, In: Stem Cells and Development. 22, 4, p. 595-610 16 p.Research output: Contribution to journal › Article › peer-review
Open Access39 Scopus citations -
Gout-causing Q141K mutation in ABCG2 leads to instability of the nucleotide-binding domain and can be corrected with small molecules
Woodward, O. M., Tukaye, D. N., Cui, J., Greenwell, P., Constantoulakis, L. M., Parker, B. S., Rao, A., Köttgen, M., Maloney, P. C. & Guggino, W. B., Mar 26 2013, In: Proceedings of the National Academy of Sciences of the United States of America. 110, 13, p. 5223-5228 6 p.Research output: Contribution to journal › Article › peer-review
Open Access76 Scopus citations -
Insulin-Like Growth Factor 1 (IGF-1) Enhances the Protein Expression of CFTR
Lee, H. W., Cheng, J., Kovbasnjuk, O., Donowitz, M. & Guggino, W. B., Mar 28 2013, In: PloS one. 8, 3, e59992.Research output: Contribution to journal › Article › peer-review
Open Access12 Scopus citations -
The GAP portion of pseudomonas aeruginosa type III secreted toxin ExoS upregulates total and surface levels of wild type CFTR
Tukaye, D. N., Kwon, S. H. & Guggino, W. B., 2013, In: Cellular Physiology and Biochemistry. 31, 1, p. 153-165 13 p.Research output: Contribution to journal › Article › peer-review
Open Access2 Scopus citations -
Transcomplementation by a truncation mutant of cystic fibrosis transmembrane conductance regulator (CFTR) EnhancesδF508 processing through a biomolecular interaction
Cebotaru, L., Woodward, O., Cebotaru, V. & Guggino, W. B., Apr 12 2013, In: Journal of Biological Chemistry. 288, 15, p. 10505-10512 8 p.Research output: Contribution to journal › Article › peer-review
Open Access10 Scopus citations -
Ubiquitination and Degradation of CFTR by the E3 Ubiquitin Ligase MARCH2 through Its Association with Adaptor Proteins CAL and STX6
Cheng, J. & Guggino, W., Jun 20 2013, In: PloS one. 8, 6, e68001.Research output: Contribution to journal › Article › peer-review
Open Access30 Scopus citations -
2012
Anion Channels
Woodward, O. M. & Guggino, W. B., Dec 31 2012, Seldin and Giebisch's The Kidney: Physiology and Pathophysiology. Elsevier, p. 1019-1045 27 p.Research output: Chapter in Book/Report/Conference proceeding › Chapter
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Dexamethasone Regulates CFTR Expression in Calu-3 Cells with the Involvement of Chaperones HSP70 and HSP90
Prota, L. F. M., Cebotaru, L., Cheng, J., Wright, J., Vij, N., Morales, M. M. & Guggino, W. B., Dec 13 2012, In: PloS one. 7, 12, e47405.Research output: Contribution to journal › Article › peer-review
Open Access17 Scopus citations -
Future directions in early cystic fibrosis lung disease research: An NHLBI workshop report
Ramsey, B. W., Banks-Schlegel, S., Accurso, F. J., Boucher, R. C., Cutting, G. R., Engelhardt, J. F., Guggino, W. B., Karp, C. L., Knowles, M. R., Kolls, J. K., LiPuma, J. J., Lynch, S., McCray, P. B., Rubenstein, R. C., Singh, P. K., Sorscher, E. & Welsh, M., Apr 15 2012, In: American journal of respiratory and critical care medicine. 185, 8, p. 887-892 6 p.Research output: Contribution to journal › Article › peer-review
56 Scopus citations -
2011
(Na + K +)-ATPase is a target for phosphoinositide 3-kinase/ protein kinase B and protein kinase C pathways triggered by albumin
Peruchetti, D. B., Pinheiro, A. A. S., Landgraf, S. S., Wengert, M., Takiya, C. M., Guggino, W. B. & Caruso-Neves, C., Dec 30 2011, In: Journal of Biological Chemistry. 286, 52, p. 45041-45047 7 p.Research output: Contribution to journal › Article › peer-review
Open Access18 Scopus citations -
A novel role of protein tyrosine kinase2 in mediating chloride secretion in human airway epithelial cells
Liang, L., Woodward, O. M., Chen, Z., Cotter, R. & Guggino, W. B., 2011, In: PloS one. 6, 7, e21991.Research output: Contribution to journal › Article › peer-review
Open Access8 Scopus citations -
CFTR Folding Consortium: Methods Available for Studies of CFTR Folding and Correction
Peters, K. W., Okiyoneda, T., Balch, W. E., Braakman, I., Brodsky, J. L., Guggino, W. B., Penland, C. M., Pollard, H. B., Sorscher, E. J., Skach, W. R., Thomas, P. J., Lukacs, G. L. & Frizzell, R. A., 2011, Methods in Molecular Biology. Humana Press Inc., p. 335-353 19 p. (Methods in Molecular Biology; vol. 742).Research output: Chapter in Book/Report/Conference proceeding › Chapter
Open Access -
Methods to Monitor Cell Surface Expression and Endocytic Trafficking of CFTR in Polarized Epithelial Cells
Bomberger, J. M., Guggino, W. B. & Stanton, B. A., 2011, Methods in Molecular Biology. Humana Press Inc., p. 271-283 13 p. (Methods in Molecular Biology; vol. 741).Research output: Chapter in Book/Report/Conference proceeding › Chapter
Open Access -
Polycystin-1, 2, and STIM1 interact with IP 3 R to Modulate ER Ca 2+ release through the PI3K/Akt pathway
Santoso, N. G., Cebotaru, L. & Guggino, W. B., 2011, In: Cellular Physiology and Biochemistry. 27, 6, p. 715-726 12 p.Research output: Contribution to journal › Article › peer-review
Open Access46 Scopus citations -
2010
Cadmium impairs albumin reabsorption by down-regulating megalin and clc5 channels in renal proximal tubule cells
Gena, P., Calamita, G. & Guggino, W. B., Nov 2010, In: Environmental health perspectives. 118, 11, p. 1551-1556 6 p.Research output: Contribution to journal › Article › peer-review
Open Access25 Scopus citations -
Drosophila TRPA1 channel is required to avoid the naturally occurring insect repellent citronellal
Kwon, Y., Kim, S. H., Ronderos, D. S., Lee, Y., Akitake, B., Woodward, O. M., Guggino, W. B., Smith, D. P. & Montell, C., 2010, In: Current Biology. 20, 18, p. 1672-1678 7 p.Research output: Contribution to journal › Article › peer-review
Open Access115 Scopus citations