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790 McCallum Building 1918 University Boulevard Birmingham, AL 35294-0005 |
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Phone: (205) 934-7210 |
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Gregory Fleming James Cystic Fibrosis Research Center |
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CF-Related Recent Publications by UAB Investigators |
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Bartoszewski R, Rab A, Twitty G, Stevenson L, Fortenberry J, Piotrowski A, Dumanski JP, Bebok Z. The mechanism of cystic fibrosis transmembrane conductance regulator transcriptional repression during the unfolded protein response. Journal of Biological Chemistry. 283(18):12154-65, 2008.
Bates E, Miller S, Alexander M, Mazur M, Fortenberry JA, Bebok Z, Sorscher EJ, Rowe SM. Bioelectric effects of quinine on polarized airway epithelial cells. Journal of Cystic Fibrosis. 6(5):351-9, 2007.
Bebok Z, Collawn JF, Wakefield J, Parker W, Li Y, Varga K, Sorscher EJ, Clancy JP. Failure of cAMP agonists to activate rescued ΔF508 CFTR in CFBE41o- in airway epithelial monolayers. Journal of Physiology. 569(Pt 2):601-15, 2005. (Cover article)
Berdiev BK, Cormet-Boyaka E, Tousson A, Qadri YJ, Oosterveld-Hut HM, Hong JS, Gonzales PA, Fuller CM, Sorscher EJ, Lukacs GL, Benos DJ. Molecular proximity of cystic fibrosis transmembrane conductance regulator and epithelial sodium channel assessed by fluorescence resonance energy transfer. Journal of Biological Chemistry. 282(50):36481-8, 2007.
Chen L, Patel RP, Teng X, Bosworth CA, Lancaster JR Jr., Matalon S. Mechanisms of cystic fibrosis transmembrane conductance regulator activation by S-nitrosoglutathione. Journal of Biological Chemistry. 281(14):9190-9, 2006.
Clancy JP, Rowe SM, Bebok Z, Aitken ML, Gibson R, Zeitlin P, Berclaz P, Moss R, Knowles MR, Oster RA, Mayer-Hamblett N, Ramsey B. No detectable improvements in cystic fibrosis transmembrane conductance regulator by nasal aminoglycosides in patients with cystic fibrosis with stop mutations. American Journal of Respiratory Cell & Molecular Biology. 37(1):57-66, 2007.
Cormet-Boyaka E, Hong JS, Berdiev BK, Fortenberry J, Rennolds J, Clancy JP, Benos DJ, Boyaka PN, Sorscher EJ. A truncated CFTR gene rescues endogenous ∆F508-CFTR and corrects chloride transport in CF mice. In preparation, 2008.
Du M, Keeling KM, Fan L, Liu X, Kovacs T, Sorscher E, Bedwell DM. Clinical doses of amikacin provide more effective suppression of the human CFTR-G542X stop mutation than gentamicin in a transgenic CF mouse model. Journal of Molecular Medicine. 84(7):573-82, 2006.
Du M, Liu X, Welch EM, Hirawat S, Peltz SW, Bedwell D. PTC124 is an orally bioavailable compound that promotes suppression of the human CFTR-G542X nonsense allele in a CF mouse model. Proceedings of the National Academy of Sciences USA. 105(6): 2064-9, 2008.
Fan-Minogue H, Bedwell DM. Eukaryotic ribosomal RNA determinants of aminoglycoside resistance and their role in translational fidelity. RNA. 14(1):148-57, 2008.
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