Zsuzsanna Bebok

Summary

Affiliation: University of Alabama at Birmingham
Country: USA

Publications

  1. doi request reprint The mechanism of cystic fibrosis transmembrane conductance regulator transcriptional repression during the unfolded protein response
    Rafal Bartoszewski
    Department of Cell Biology, University of Alabama, Birmingham, Alabama 35294 0005, USA
    J Biol Chem 283:12154-65. 2008
  2. ncbi request reprint Endoplasmic reticulum stress and the unfolded protein response regulate genomic cystic fibrosis transmembrane conductance regulator expression
    Andras Rab
    Dept of Cell Biology, Univ of Alabama at Birmingham, 1918 Univ Blvd, MCLM 760, Birmingham, AL 35294 0005, USA
    Am J Physiol Cell Physiol 292:C756-66. 2007
  3. pmc Interaction of ASIC1 and ENaC subunits in human glioma cells and rat astrocytes
    Niren Kapoor
    Dept of Physiology and Biophysics, University of Alabama at Birmingham, 1918 University Blvd, Birmingham, AL 35294, USA
    Am J Physiol Cell Physiol 300:C1246-59. 2011
  4. pmc Targets for cystic fibrosis therapy: proteomic analysis and correction of mutant cystic fibrosis transmembrane conductance regulator
    James F Collawn
    University of Alabama at Birmingham, Department of Cell Biology, Birmingham, AL, USA
    Expert Rev Proteomics 7:495-506. 2010
  5. pmc Restoration of W1282X CFTR activity by enhanced expression
    Steven M Rowe
    Department of Medicine, Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, Alabama 35294 0006, USA
    Am J Respir Cell Mol Biol 37:347-56. 2007
  6. pmc No detectable improvements in cystic fibrosis transmembrane conductance regulator by nasal aminoglycosides in patients with cystic fibrosis with stop mutations
    John P Clancy
    Department of Pediatrics, University of Alabama at Birmingham, 1600 7th Avenue South, Birmingham, AL 35233, USA
    Am J Respir Cell Mol Biol 37:57-66. 2007
  7. pmc Failure of cAMP agonists to activate rescued deltaF508 CFTR in CFBE41o- airway epithelial monolayers
    Zsuzsa Bebok
    Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, AL 35233, USA
    J Physiol 569:601-15. 2005
  8. ncbi request reprint Extracellular zinc and ATP restore chloride secretion across cystic fibrosis airway epithelia by triggering calcium entry
    Akos Zsembery
    Department of Physiology and Biophysics, Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama, Birmingham 35294 0005, USA
    J Biol Chem 279:10720-9. 2004
  9. ncbi request reprint Reactive oxygen nitrogen species decrease cystic fibrosis transmembrane conductance regulator expression and cAMP-mediated Cl- secretion in airway epithelia
    Zsuzsa Bebok
    Department of Medicine, and The Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama, Birmingham, Alabama 35233, USA
    J Biol Chem 277:43041-9. 2002
  10. pmc Activation of the unfolded protein response by deltaF508 CFTR
    Rafal Bartoszewski
    Department of Cell Biology, University of Alabama at Birmingham, Birmingham, AL 35294 0005, USA
    Am J Respir Cell Mol Biol 39:448-57. 2008

Research Grants

  1. CFTR Biogenesis and Function in Epithelia
    Zsuzsanna Bebok; Fiscal Year: 2007
  2. CFTR Biogenesis and Function in Epithelia
    Zsuzsanna Bebok; Fiscal Year: 2009
  3. CFTR Biogenesis and Function in Epithelia
    Zsuzsa Bebok; Fiscal Year: 2010

Collaborators

  • James Collawn
  • John P Clancy
  • S M Rowe
  • ERIK MILLS SCHWIEBERT
  • Nicole Mayer-Hamblett
  • Lianwu Fu
  • Bonnie W Ramsey
  • William Parker
  • Michael R Knowles
  • Helen Kim
  • Akos Zsembery
  • Margarida D Amaral
  • A Dragomir
  • Charles J Venglarik
  • Rafal Bartoszewski
  • Asta Jurkuvenaite
  • Andras Rab
  • Eric J Sorscher
  • Rebecca F Goldstein
  • Karoly Varga
  • Niren Kapoor
  • Rafal A Bartoszewski
  • Sadis Matalon
  • Lauren Stevenson
  • Lan Chen
  • John Wakefield
  • Marina Mazur
  • Eleanor Bates
  • Daniel Klink
  • Ceinwen M Harris
  • Krisztina Peter
  • Vladimir Parpura
  • Carmel M McNicholas
  • Cheryl A Palmer
  • Edlira Clark
  • William Lee
  • Cecelia B Latham
  • Catherine M Fuller
  • Dale J Benos
  • Qun Dai
  • Michael Jablonsky
  • John Kappes
  • Rebecca Goldstein
  • Sylwia Bartoszewska
  • Arkadiusz Piotrowski
  • Jan P Dumanski
  • James Fortenberry
  • George Twitty
  • Ashutosh Niraj
  • Stacey Miller
  • Todd P Sanderson
  • Mariah Alexander
  • James A Fortenberry
  • Landon S Wilson
  • Bob J Scholte
  • Margaret A McPherson
  • A Christopher Boyd
  • Filipa Mendes
  • I Carvalho-Oliveira
  • Robert L Dormer
  • Dirk Schindelhauer
  • Valerie Eubanks
  • Torry Tucker
  • Deborah Penque
  • Andreas Laner
  • Iolo J M Doull
  • Godfried M Roomans
  • Carmel M McNicholas-Bevensee
  • Lisa Schwiebert

Detail Information

Publications18

  1. doi request reprint The mechanism of cystic fibrosis transmembrane conductance regulator transcriptional repression during the unfolded protein response
    Rafal Bartoszewski
    Department of Cell Biology, University of Alabama, Birmingham, Alabama 35294 0005, USA
    J Biol Chem 283:12154-65. 2008
    ..These studies provide novel insight into the mechanism of gene repression during the mammalian UPR...
  2. ncbi request reprint Endoplasmic reticulum stress and the unfolded protein response regulate genomic cystic fibrosis transmembrane conductance regulator expression
    Andras Rab
    Dept of Cell Biology, Univ of Alabama at Birmingham, 1918 Univ Blvd, MCLM 760, Birmingham, AL 35294 0005, USA
    Am J Physiol Cell Physiol 292:C756-66. 2007
    ....
  3. pmc Interaction of ASIC1 and ENaC subunits in human glioma cells and rat astrocytes
    Niren Kapoor
    Dept of Physiology and Biophysics, University of Alabama at Birmingham, 1918 University Blvd, Birmingham, AL 35294, USA
    Am J Physiol Cell Physiol 300:C1246-59. 2011
    ..These data suggest that different ENaC/Deg subunits interact and could combine to form a hybrid channel that likely underlies the amiloride-sensitive current seen in human glioma cells...
  4. pmc Targets for cystic fibrosis therapy: proteomic analysis and correction of mutant cystic fibrosis transmembrane conductance regulator
    James F Collawn
    University of Alabama at Birmingham, Department of Cell Biology, Birmingham, AL, USA
    Expert Rev Proteomics 7:495-506. 2010
    ....
  5. pmc Restoration of W1282X CFTR activity by enhanced expression
    Steven M Rowe
    Department of Medicine, Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, Alabama 35294 0006, USA
    Am J Respir Cell Mol Biol 37:347-56. 2007
    ..These findings provide evidence that W1282X CFTR exhibits membrane localization and retained chloride channel function after enhanced expression, and suggest that patients harboring this mutation may be more susceptible to CFTR rescue...
  6. pmc No detectable improvements in cystic fibrosis transmembrane conductance regulator by nasal aminoglycosides in patients with cystic fibrosis with stop mutations
    John P Clancy
    Department of Pediatrics, University of Alabama at Birmingham, 1600 7th Avenue South, Birmingham, AL 35233, USA
    Am J Respir Cell Mol Biol 37:57-66. 2007
    ..The study provides valuable information on parameters of the nasal potential difference measurements for use in future multicenter clinical trials...
  7. pmc Failure of cAMP agonists to activate rescued deltaF508 CFTR in CFBE41o- airway epithelial monolayers
    Zsuzsa Bebok
    Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, AL 35233, USA
    J Physiol 569:601-15. 2005
    ....
  8. ncbi request reprint Extracellular zinc and ATP restore chloride secretion across cystic fibrosis airway epithelia by triggering calcium entry
    Akos Zsembery
    Department of Physiology and Biophysics, Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama, Birmingham 35294 0005, USA
    J Biol Chem 279:10720-9. 2004
    ..Our data suggest that activation of P2X calcium entry channels may have profound therapeutic benefit for CF that is independent of cystic fibrosis transmembrane conductance regulator genotype...
  9. ncbi request reprint Reactive oxygen nitrogen species decrease cystic fibrosis transmembrane conductance regulator expression and cAMP-mediated Cl- secretion in airway epithelia
    Zsuzsa Bebok
    Department of Medicine, and The Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama, Birmingham, Alabama 35233, USA
    J Biol Chem 277:43041-9. 2002
    ..Decreased levels and function of normal CFTR may account for some of the cystic fibrosis-like symptoms that occur in chronic inflammatory lung diseases associated with increased NO production...
  10. pmc Activation of the unfolded protein response by deltaF508 CFTR
    Rafal Bartoszewski
    Department of Cell Biology, University of Alabama at Birmingham, Birmingham, AL 35294 0005, USA
    Am J Respir Cell Mol Biol 39:448-57. 2008
    ....
  11. pmc Bioelectric effects of quinine on polarized airway epithelial cells
    Eleanor Bates
    Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, 1900 University Boulevard, Birmingham, AL 35294, USA
    J Cyst Fibros 6:351-9. 2007
    ..The use of quinine as a taste-masking agent may have bioelectric effects relevant to CF trials using aerosolized drug delivery...
  12. pmc A synonymous single nucleotide polymorphism in DeltaF508 CFTR alters the secondary structure of the mRNA and the expression of the mutant protein
    Rafal A Bartoszewski
    Department of Cell Biology, University of Alabama at Birmingham, Birmingham, Alabama 35294 0005, USA
    J Biol Chem 285:28741-8. 2010
    ..Our studies suggest that in addition to modifier genes, SNPs may also contribute to the differences observed in the symptoms of various DeltaF508 homozygous CF patients...
  13. ncbi request reprint Enhanced cell-surface stability of rescued DeltaF508 cystic fibrosis transmembrane conductance regulator (CFTR) by pharmacological chaperones
    Karoly Varga
    Department of Cell Biology, University of Alabama at Birmingham, Birmingham, AL 35294, USA
    Biochem J 410:555-64. 2008
    ..The results indicate that small molecular correctors may reproduce the effect of incubation at the permissive temperature, not only by rescuing DeltaF508 CFTR from ERAD, but also by enhancing its cell-surface stability...
  14. pmc VCP/p97 AAA-ATPase does not interact with the endogenous wild-type cystic fibrosis transmembrane conductance regulator
    Rebecca F Goldstein
    Department of Cell Biology, Comprehensive Cancer Center, and Gregory Fleming James Cystic Fibrosis Center, University of Alabama at Birmingham, Birmingham, Alabama 35294 0005, USA
    Am J Respir Cell Mol Biol 36:706-14. 2007
    ....
  15. pmc Functional stability of rescued delta F508 cystic fibrosis transmembrane conductance regulator in airway epithelial cells
    Asta Jurkuvenaite
    Department of Cell Biology, University of Alabama at Birmingham, Birmingham, AL 35294, USA
    Am J Respir Cell Mol Biol 42:363-72. 2010
    ....
  16. ncbi request reprint Ablation of internalization signals in the carboxyl-terminal tail of the cystic fibrosis transmembrane conductance regulator enhances cell surface expression
    Krisztina Peter
    Department of Cell Biology, Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, 1918 University Boulevard, Birmingham, AL 35294 0005, USA
    J Biol Chem 277:49952-7. 2002
    ..This signal regulates CFTR surface expression but not CFTR biogenesis, degradation, or chloride channel function...
  17. ncbi request reprint Assessment of CFTR localisation in native airway epithelial cells obtained by nasal brushing
    Ceinwen M Harris
    Department of Medical Biochemistry and Immunology, University of Wales College of Medicine, Heath Park, Cardiff, Wales CF14 4XN, UK
    J Cyst Fibros 3:43-8. 2004
    ..The protocols are a consensus, following comparison of some aspects of methods currently used in the authors' laboratories...
  18. ncbi request reprint Gene delivery systems--gene therapy vectors for cystic fibrosis
    Daniel Klink
    Department of Cell Biology, Erasmus MC, Erasmus University, P O Box 1738, Rotterdam DR 3000, The Netherlands
    J Cyst Fibros 3:203-12. 2004
    ..The intact airway epithelium, the most important target in CF gene therapy, proves to be well protected against invading vector systems...

Research Grants6

  1. CFTR Biogenesis and Function in Epithelia
    Zsuzsanna Bebok; Fiscal Year: 2007
    ..Completion of these experiments will provide novel and important information on a physiologically important, multi-domain; integral membrane protein is synthesized in a number of epithelial cell types. ..
  2. CFTR Biogenesis and Function in Epithelia
    Zsuzsanna Bebok; Fiscal Year: 2009
    ..Understanding these processes are important in understanding the cellular basis for a number of lung pathologies including cystic fibrosis and chronic obstructive pulmonary disease (COPD). ..
  3. CFTR Biogenesis and Function in Epithelia
    Zsuzsa Bebok; Fiscal Year: 2010
    ..Understanding these processes are important in understanding the cellular basis for a number of lung pathologies including cystic fibrosis and chronic obstructive pulmonary disease (COPD). ..