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Faculty & Research

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William Guggino

Department Affiliation Primary: Physiology
Secondary: Pediatrics
RankProfessor
Phone Numbers(410) 955-7166
Fax: (410) 955-0461
Emailwguggino@jhmi.edu
School of Medicine Address725 N. Wolfe St.
Physiology 202A
Baltimore MD 21219
Link to Lab Homepage
William Guggino

Research Topic: 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.

Publications:

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.
PubMed Reference

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 ƒ´F508-CFTR cell-surface expression: an RNA interference and structure-based mutagenetic approach. J Biol Chem. 282, 8099-8109.
PubMed Reference

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.
PubMed Reference

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.
PubMed Reference

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.
PubMed Reference

Guggino WB & Stanton BA. (2006). New insights into cystic fibrosis: molecular switches that regulate CFTR. Nat Rev Mol Cell Biol 7, 426-436.
PubMed Reference

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.
PubMed Reference

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.
PubMed Reference

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.
PubMed Reference

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.
PubMed Reference

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.
PubMed Reference

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​

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