·         Hara-Chikuma M, Wang Y, Guggino SE, Guggino WB, & Verkman AS (2005). Impaired acidification in early endosomes of ClC-5 deficient proximal tubule. Biochem Biophys Res Commun 329, 941-946.

·         Lewis HA, Zhao X, Wang C, Sauder JM, Rooney I, Noland BW, Lorimer D, Kearins MC, Conners K, Condon B, Maloney PC, Guggino WB, Hunt JF, & Emtage S (2005). Impact of the DeltaF508 Mutation in First Nucleotide-binding Domain of Human Cystic Fibrosis Transmembrane Conductance Regulator on Domain Folding and Structure. J Biol Chem 280, 1346-1353.

·         Li Y, Wright JM, Qian F, Germino GG, & Guggino WB (2005). Polycystin 2 interacts with type I inositol 1,4,5-trisphosphate receptor to modulate intracellular Ca2+ signaling. J Biol Chem 280, 41298-41306.

·         Rebelo MA, Tostes V, Araujo NC, Martini SV, Botelho BF, Guggino WB, & Morales MM (2005). Screening for CLCN5 mutation in renal calcium stone formers patients. An Acad Bras Cienc 77, 95-101.

·         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.

·         Cheng J, Wang H, & Guggino WB (2004). Modulation of mature cystic fibrosis transmembrane regulator protein by the PDZ domain protein CAL. J Biol Chem 279, 1892-1898.

·         Chikhlikar P, Barros de AL, Agrawal S, Byrne B, Guggino W, August JT, & Marques ET, Jr. (2004). Inverted terminal repeat sequences of adeno-associated virus enhance the antibody and CD8(+) responses to a HIV-1 p55Gag/LAMP DNA vaccine chimera. Virology 323, 220-232.

·         Guggino WB & Banks-Schlegel SP (2004). Macromolecular interactions and ion transport in cystic fibrosis. Am J Respir Crit Care Med 170, 815-820.

·         Guggino WB (2004). The cystic fibrosis transmembrane regulator forms macromolecular complexes with PDZ domain scaffold proteins. Proc Am Thorac Soc 1, 28-32.

·         Hryciw DH, Ekberg J, Lee A, Lensink IL, Kumar S, Guggino WB, Cook DI, Pollock CA, & Poronnik P (2004). Nedd4-2 functionally interacts with ClC-5: involvement in constitutive albumin endocytosis in proximal tubule cells. J Biol Chem.279, 54996-55007.

·         Karp CL, Flick LM, Park KW, Softic S, Greer TM, Keledjian R, Yang R, Uddin J, Guggino WB, Atabani SF, Belkaid Y, Xu Y, Whitsett JA, Accurso FJ, Wills-Karp M, & Petasis NA (2004). Defective lipoxin-mediated anti-inflammatory activity in the cystic fibrosis airway. Nat Immunol 5, 388-392.

·         Kwon SH & Guggino WB (2004). Multiple sequences in the C terminus of MaxiK channels are involved in expression, movement to the cell surface, and apical localization. Proc Natl Acad Sci U S A. 101, 15237-15242.

·         Lewis HA, Buchanan SG, Burley SK, Conners K, Dickey M, Dorwart M, Fowler R, Gao X, Guggino WB, Hendrickson WA, Hunt JF, Kearins MC, Lorimer D, Maloney PC, Post KW, Rajashankar KR, Rutter ME, Sauder JM, Shriver S, Thibodeau PH, Thomas PJ, Zhang M, Zhao X, & Emtage S (2004). Structure of nucleotide-binding domain 1 of the cystic fibrosis transmembrane conductance regulator. EMBO J 23, 282-293.

·         Sirninger J, Muller C, Braag S, Tang Q, Yue H, Detrisac C, Ferkol T, Guggino WB, & Flotte TR (2004). Functional characterization of a recombinant adeno-associated virus 5-pseudotyped cystic fibrosis transmembrane conductance regulator vector. Hum Gene Ther 15, 832-841.

·         Wright JM, Zeitlin PL, Cebotaru L, Guggino SE, & Guggino WB (2004). Gene expression profile analysis of 4-phenylbutyrate treatment of IB3-1 bronchial epithelial cell line demonstrates a major influence on heat-shock proteins. Physiol Genomics 16, 204-211.

·         Zeitlin PL, Boyle MP, Guggino WB, & Molina L (2004). A phase I trial of intranasal Moli1901 for cystic fibrosis. Chest 125, 143-149.

·         Boyle MP, Enke RA, Mogayzel PJ, Jr., Guggino WB, Martin DB, Agarwal S, & Zeitlin PL (2003). Effect of adeno-associated virus-specific immunoglobulin G in human amniotic fluid on gene transfer. Hum Gene Ther 14, 365-373.

·         Christensen EI, Devuyst O, Dom G, Nielsen R, Van der SP, Verroust P, Leruth M, Guggino WB, & Courtoy PJ (2003). Loss of chloride channel ClC-5 impairs endocytosis by defective trafficking of megalin and cubilin in kidney proximal tubules. Proc Natl Acad Sci U S A 100, 8472-8477.

·         Fischer AC, Beck SE, Smith CI, Laube BL, Askin FB, Guggino SE, Adams RJ, Flotte TR, & Guggino WB (2003). Successful transgene expression with serial doses of aerosolized rAAV2 vectors in rhesus macaques. Mol Ther 8, 918-926.

·         Flotte TR, Zeitlin PL, Reynolds TC, Heald AE, Pedersen P, Beck S, Conrad CK, Brass-Ernst L, Humphries M, Sullivan K, Wetzel R, Taylor G, Carter BJ, & Guggino WB (2003). Phase I trial of intranasal and endobronchial administration of a recombinant adeno-associated virus serotype 2 (rAAV2)-CFTR vector in adult cystic fibrosis patients: a two-part clinical study. Hum Gene Ther 14, 1079-1088.

·         Fong P, Argent BE, Guggino WB, & Gray MA (2003). Characterization of vectorial chloride transport pathways in the human pancreatic duct adenocarcinoma cell line HPAF. Am J Physiol Cell Physiol 285, C433-C445.

·         Hryciw DH, Wang Y, Devuyst O, Pollock CA, Poronnik P, & Guggino WB (2003). Cofilin interacts with ClC-5 and regulates albumin uptake in proximal tubule cell lines. J Biol Chem 278, 40169-40176.

·         Nascimento DS, Reis CU, Goldenberg RC, Ortiga-Carvalho TM, Pazos-Moura CC, Guggino SE, Guggino WB, & Morales MM (2003). Estrogen modulates ClC-2 chloride channel gene expression in rat kidney. Pflugers Arch 446, 593-599.

·         Santos OD, Grozovsky R, Goldenberg RC, Carvalho DP, Fong P, Guggino WB, & Morales M (2003). Thyroid hormone modulates ClC-2 chloride channel gene expression in rat renal proximal tubules. J Endocrinol 178, 503-511.

·         Silva IV, Cebotaru V, Wang H, Wang XT, Wang SS, Guo G, Devuyst O, Thakker RV, Guggino WB, & Guggino SE (2003). The ClC-5 knockout mouse model of Dent's disease has renal hypercalciuria and increased bone turnover. J Bone Miner Res 18, 615-623.

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