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Professor and Director
Director, Department of Physiology
725 N. Wolfe St.
Baltimore MD 21219
Genetic disorders associated with defective fluid and electrolyte movement; ion channel and epithelial transport physiology
Endoplasmic Reticulum (ER) is the largest intracellular Ca 2+ store. On the membrane of the ER, there are calcium release components such as inositol 1, 4, 5 trisphosphate receptor (IP 3 R), and the calcium pumps like SR/ER Ca 2+ ATPases (SERCAs). We injected caged-IP 3 together with the fluorescent Ca 2+ indicator into the oocytes. Upon UV stimulation, activated IP 3 binds to IP 3 R and releases Ca 2+ from the ER into the cytosol. Subsequently the closure of IP 3 R and activation of SERCAs takes the cytosolic Ca 2+ back into the ER stores. We recorded the changes of intracellular Ca 2+ signals by confocal microscopy. The movie shows the different intracellular calcium responses induced by UV-uncaging of caged IP 3 in Xenopus oocytes overexpressing polycystin-2 and the water injected control.
Cebotaru L and Guggino WB. Complement yourself: Transcomplementation rescues partially folded mutant proteins. BiophysRev 6: 169-180, 2014.
Cebotaru L, Liu Q, Yanda MK, Boinot C, Outeda P, Huso DL, Watnick T, Guggino WB, and Cebotaru V. Inhibition of histone deacetylase 6 activity reduces cyst growth in polycystic kidney disease. Kidney international 90: 90-99, 2016.
Lopes‐Pacheco M, Sabirzhanova I, Rapino D, Morales MM, Guggino WB, and Cebotaru L. Correctors Rescue CFTR Mutations in Nucleotide‐Binding Domain 1 (NBD1) by Modulating Proteostasis. ChemBioChem 17: 493-505, 2016.
Santoso NG, Cebotaru L, and Guggino WB. Polycystin-1, 2, and STIM1 interact with IP(3)R to modulate ER Ca release through the PI3K/Akt pathway. Cell Physiol Biochem 27: 715-726, 2011.
Wang H, Cebotaru L, Lee H, Yang Q, Pollard B, Pollard H, and Guggino W. CFTR Controls the Activity of NF-κB by Enhancing the Degradation of TRADD. cell physiol biochem 40: 1063-1078, 2016.
Woodward OM, Tukaye DN, Cui J, Greenwell P, Constantoulakis LM, Parker BS, Rao A, Kottgen M, Maloney PC, and Guggino WB. Gout-causing Q141K mutation in ABCG2 leads to instability of the nucleotide-binding domain and can be corrected with small molecules. Proc Natl Acad Sci U S A 110: 5223-5228, 2013.
Fischer AC, Smith CI, Cebotaru L, Zhang X, Askin FB, Wright J, Guggino SE, Adams RJ, Flotte T, & Guggino WB. (2007). Expression of a Truncated Cystic Fibrosis Transmembrane Conductance Regulator with an AAV5-pseudotyped Vector in Primates. Mol Ther 15, 756-763.
Wolde M, Fellows A, Cheng J, Kivenson A, Coutermarsh B, Talebian L, Karlson K, Piserchio A, Mierke DF, Stanton BA, Guggino WB, & Madden DR. (2007). Targeting CAL as a negative regulator of ѓґF508-CFTR cell-surface expression: an RNA interference and structure-based mutagenetic approach. J Biol Chem. 282, 8099-8109.
Beitz E, Liu K, Ikeda M, Guggino WB, Agre P, & Yasui M. (2006). Determinants of AQP6 trafficking to intracellular sites vs. to the plasma membrane in transfected mammalian cells. Biol Cell. 98, 101-109.
Boyle MP, Enke RA, Reynolds JB, Mogayzel PJ, Jr., Guggino WB, & Zeitlin PL (2006). Membrane-associated heparan sulfate is not required for rAAV-2 infection of human respiratory epithelia. Virol J 3, 29-36.
Cai H, Cebotaru V, Wang YH, Zhang XM, Cebotaru L, Guggino SE, & Guggino WB. (2006). WNK4 kinase regulates surface expression of the human sodium chloride cotransporter in mammalian cells. Kidney Int 69, 2162-2170.
Guggino WB & Stanton BA. (2006). New insights into cystic fibrosis: molecular switches that regulate CFTR. Nat Rev Mol Cell Biol 7, 426-436.
Ikeda M, Fon, P, Cheng J, Boletta A, Qian F, Zhang XM, Cai H, Germino GG, & Guggino WB. (2006). A regulatory role of polycystin-1 on cystic fibrosis transmembrane conductance regulator plasma membrane expression. Cell Physiol. Biochem. 18, 9-20.
Novaira HJ, Ornellas DS, Ortiga-Carvalho TM, Zhang XM, Souza-Menezes J, Guggino SE, Guggino WB, & Morales MM. (2006). Atrial natriuretic peptide modulates cystic fibrosis transmembrane conductance regulator chloride channel expression in rat proximal colon and human intestinal epithelial cells. J Endocrinol 189, 155-165.
Pollard HB, Eidelman O, Jozwik C, Huang W, Srivastava M, Ji XD. McGowan B, Formas NC, Todo S, Darling T, Mogayze, PJ, Zeitlin PL, Wright J, Guggino WB, Metcalf E, Driscoll W, Mueller G, Paweletz C, & Jacobowitz DM. (2006). de Novo biosynthetic profiling of high abundance proteinsin cystic fibrosis lung epithelial cells. Mol Cell Proteomics 5, 1628-1637.
van den Hove MF, Croizet-Berger K, Jouret F, Guggino SE, Guggino WB, Devuyst, O, & Courtoy PJ. (2006). The loss of the chloride channel, ClC-5, delays apical iodide efflux and induces an euthyroid goiter in the mouse thyroid gland. Endocrinology. 147, 1287-1296.
Wright JM, Merlo CA, Reynolds JB, Zeitlin PL, Garcia JG, Guggino WB, & Boyle MP. (2006). Respiratory Epithelial Gene Expression in Cystic Fibrosis Patients with Mild and Severe Lung Disease. Am J Respir Cell Mol Biol. 35, 327-336.
Wang Y, Cai H, Cebotaru L, Hryciw DH, Weinman EJ, Donowitz M. Guggino SE, & Guggino WB. (2005). ClC-5: role in endocytosis in the proximal tubule. Am J Physiol. Renal Physiol. 289, F850-F862. PubMed Reference?