Positions

Overview

  • MAJOR RESEARCH INTERESTS
    1) Membrane protein biogenesis in epithelial cells
    a. Endoplasmic reticulum-associated degradation (ERAD)
    b. ER quality control (EQC)
    c. The unfolded protein response (UPR)
    2) Gene expression regulation by the UPR
    a. The mechanisms of UPR-associated transcriptional repression
    b. The mechanisms of the UPR-activated ERAD
    c. UPR activated micro-RNAs
    d. The role of miRNAs in the regulation of ER protein influx during the UPR.
    e. The role of cell-non-autonomous UPR in gene expression regulation
    3) The role of synonymous mutations in determination of protein structure and function. Regulation of membrane protein translation and cotranslational folding by mRNA secondary structure alterations.
    4) UPR-mediated pathways of polycystic kidney disease pathogenesis. I am collaborating with Drs. Bradly Yoder and Darwin Bell to investigate how cellular stress responses contribute to the development of polycystic kidney disease.
    5) UPR-mediated pathways of chronic respiratory disorders.
  • Selected Publications

    Academic Article

    Year Title Altmetric
    2017 Influenza virus infection alters ion channel function of airway and alveolar cells: mechanisms and physiological sequelae.AJP - Lung Cellular and Molecular Physiology.  313:L845-L858. 2017
    2017 miR-200b downregulates CFTR during hypoxia in human lung epithelial cells.Cellular and Molecular Biology Letters.  22:23. 2017
    2016 A synonymous codon change alters the drug sensitivity of ΔF508 cystic fibrosis transmembrane conductance regulator.Federation proceedings.  30:201-213. 2016
    2016 Limited ATF4 Expression in Degenerating Retinas with Ongoing ER Stress Promotes Photoreceptor Survival in a Mouse Model of Autosomal Dominant Retinitis Pigmentosa.PLoS ONE.  11:e0154779. 2016
    2016 Mechanistic Approaches to Improve Correction of the Most Common Disease-Causing Mutation in Cystic Fibrosis.PLoS ONE.  11:e0155882. 2016
    2015 Decoding mechanisms by which silent codon changes influence protein biogenesis and function.The International journal of biochemistry.  64:58-74. 2015
    2015 Influenza virus M2 targets cystic fibrosis transmembrane conductance regulator for lysosomal degradation during viral infection.Federation proceedings.  29:2712-2725. 2015
    2015 ΔF508 CFTR surface stability is regulated by DAB2 and CHIP-mediated ubiquitination in post-endocytic compartments.PLoS ONE.  10:e0123131. 2015
    2013 The silent codon change I507-ATC->ATT contributes to the severity of the ΔF508 CFTR channel dysfunction.Federation proceedings.  27:4630-4645. 2013
    2013 Cigarette smoke and CFTR: implications in the pathogenesis of COPD.AJP - Lung Cellular and Molecular Physiology.  305:L530-L541. 2013
    2013 Regulation of angiogenesis by hypoxia: the role of microRNA.Cellular and Molecular Biology Letters.  18:47-57. 2013
    2012 The CFTR and ENaC debate: how important is ENaC in CF lung disease?AJP - Lung Cellular and Molecular Physiology.  302:L1141-L1146. 2012
    2012 Glioma-specific cation conductance regulates migration and cell cycle progression.Journal of Biological Chemistry.  287:4053-4065. 2012
    2012 Dab2 is a key regulator of endocytosis and post-endocytic trafficking of the cystic fibrosis transmembrane conductance regulator.Biochemical Journal.  441:633-643. 2012
    2011 The unfolded protein response (UPR)-activated transcription factor X-box-binding protein 1 (XBP1) induces microRNA-346 expression that targets the human antigen peptide transporter 1 (TAP1) mRNA and governs immune regulatory genes.Journal of Biological Chemistry.  286:41862-41870. 2011
    2011 Suppression of CFTR premature termination codons and rescue of CFTR protein and function by the synthetic aminoglycoside NB54.The Clinical investigator.  89:1149-1161. 2011
    2011 Interaction of ASIC1 and ENaC subunits in human glioma cells and rat astrocytes.American Journal of Physiology - Cell Physiology.  300:C1246-C1259. 2011
    2011 CFTR expression regulation by the unfolded protein response.Methods in Enzymology.  491:3-24. 2011
    2010 A synonymous single nucleotide polymorphism in DeltaF508 CFTR alters the secondary structure of the mRNA and the expression of the mutant protein.Journal of Biological Chemistry.  285:28741-28748. 2010
    2010 Targets for cystic fibrosis therapy: proteomic analysis and correction of mutant cystic fibrosis transmembrane conductance regulator.Expert Review of Proteomics.  7:495-506. 2010
    2010 Functional stability of rescued delta F508 cystic fibrosis transmembrane conductance regulator in airway epithelial cells.American Journal of Respiratory Cell and Molecular Biology.  42:363-372. 2010
    2009 Knockdown of ASIC1 and epithelial sodium channel subunits inhibits glioblastoma whole cell current and cell migration.Journal of Biological Chemistry.  284:24526-24541. 2009
    2009 Search and rescue: finding ways to correct deltaF508 CFTR.American Journal of Respiratory Cell and Molecular Biology.  40:385-387. 2009
    2008 Activation of the unfolded protein response by deltaF508 CFTR.American Journal of Respiratory Cell and Molecular Biology.  39:448-457. 2008
    2008 Somatic mosaicism for copy number variation in differentiated human tissues.Human Mutation.  29:1118-1124. 2008
    2008 The mechanism of cystic fibrosis transmembrane conductance regulator transcriptional repression during the unfolded protein response.Journal of Biological Chemistry.  283:12154-12165. 2008
    2008 Enhanced cell-surface stability of rescued DeltaF508 cystic fibrosis transmembrane conductance regulator (CFTR) by pharmacological chaperones.Biochemical Journal.  410:555-564. 2008
    2007 Bioelectric effects of quinine on polarized airway epithelial cells.Journal of Cystic Fibrosis.  6:351-359. 2007
    2007 Restoration of W1282X CFTR activity by enhanced expression.American Journal of Respiratory Cell and Molecular Biology.  37:347-356. 2007
    2007 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 and Molecular Biology.  37:57-66. 2007
    2007 VCP/p97 AAA-ATPase does not interact with the endogenous wild-type cystic fibrosis transmembrane conductance regulator.American Journal of Respiratory Cell and Molecular Biology.  36:706-714. 2007
    2007 Endoplasmic reticulum stress and the unfolded protein response regulate genomic cystic fibrosis transmembrane conductance regulator expression.American Journal of Physiology - Cell Physiology.  292:C756-C766. 2007
    2006 Mutations in the amino terminus of the cystic fibrosis transmembrane conductance regulator enhance endocytosis.Journal of Biological Chemistry.  281:3329-3334. 2006
    2005 Failure of cAMP agonists to activate rescued deltaF508 CFTR in CFBE41o- airway epithelial monolayers.Journal of Physiology.  569:601-615. 2005
    2005 Consequences of C-terminal domains and N-terminal signal peptide deletions on LEKTI secretion, stability, and subcellular distribution.Archives of Biochemistry and Biophysics.  435:89-102. 2005
    2005 Establishment and characterization of a novel polarized MDCK epithelial cellular model for CFTR studies.Cellular Physiology and Biochemistry.  16:281-290. 2005
    2004 Excellent in vivo bystander activity of fludarabine phosphate against human glioma xenografts that express the escherichia coli purine nucleoside phosphorylase gene.Cancer Research.  64:6610-6615. 2004
    2004 Assessment of CFTR localisation in native airway epithelial cells obtained by nasal brushing.Journal of Cystic Fibrosis.  3 Suppl 2:43-48. 2004
    2004 Gene delivery systems--gene therapy vectors for cystic fibrosis.Journal of Cystic Fibrosis.  3 Suppl 2:203-212. 2004
    2004 Efficient intracellular processing of the endogenous cystic fibrosis transmembrane conductance regulator in epithelial cell lines.Journal of Biological Chemistry.  279:22578-22584. 2004
    2004 Extracellular zinc and ATP restore chloride secretion across cystic fibrosis airway epithelia by triggering calcium entry.Journal of Biological Chemistry.  279:10720-10729. 2004
    2003 Gentamicin-induced correction of CFTR function in patients with cystic fibrosis and CFTR stop mutations.New England Journal of Medicine.  349:1433-1441. 2003
    2003 Transient transfection of polarized epithelial monolayers with CFTR and reporter genes using efficacious lipidsAmerican Journal of Physiology - Cell Physiology.  284. 2003
    2003 Transient transfection of polarized epithelial monolayers with CFTR and reporter genes using efficacious lipids.American Journal of Physiology - Cell Physiology.  284:C791-C804. 2003
    2002 Ablation of internalization signals in the carboxyl-terminal tail of the cystic fibrosis transmembrane conductance regulator enhances cell surface expression.Journal of Biological Chemistry.  277:49952-49957. 2002
    2002 Reactive oxygen nitrogen species decrease cystic fibrosis transmembrane conductance regulator expression and cAMP-mediated Cl- secretion in airway epithelia.Journal of Biological Chemistry.  277:43041-43049. 2002
    2002 Aminoglycoside suppression of a premature stop mutation in a Cftr-/- mouse carrying a human CFTR-G542X transgene.The Clinical investigator.  80:595-604. 2002
    2002 Novel regulation of cell [Na+] in macula densa cells: Apical Na+ recycling by H-K-ATPaseAJP - Renal Physiology.  282. 2002
    2002 Novel regulation of cell [Na(+)] in macula densa cells: apical Na(+) recycling by H-K-ATPase.AJP - Renal Physiology.  282:F324-F329. 2002
    2001 Improved oxygenation promotes CFTR maturation and trafficking in MDCK monolayersAmerican Journal of Physiology - Cell Physiology.  280. 2001
    2001 Evidence that systemic gentamicin suppresses premature stop mutations in patients with cystic fibrosis.American Journal of Respiratory and Critical Care Medicine.  163:1683-1692. 2001
    2001 A clinical inflammatory syndrome attributable to aerosolized lipid-DNA administration in cystic fibrosis.Human Gene Therapy.  12:751-761. 2001
    2001 Improved oxygenation promotes CFTR maturation and trafficking in MDCK monolayers.American Journal of Physiology - Cell Physiology.  280:C135-C145. 2001
    2000 Macula densa Na(+)/H(+) exchange activities mediated by apical NHE2 and basolateral NHE4 isoforms.AJP - Renal Physiology.  278:F452-F463. 2000
    2000 Macula densa Na+/H+ exchange activities mediated by apical NHE2 and basolateral NHE4 isoformsAJP - Renal Physiology.  278. 2000
    1999 CFTR involvement in chloride, bicarbonate, and liquid secretion by airway submucosal glandsAJP - Lung Cellular and Molecular Physiology.  277. 1999
    1999 CFTR involvement in chloride, bicarbonate, and liquid secretion by airway submucosal glands.American Journal of Physiology.  277:L694-L699. 1999
    1999 Characterization and dynamics of aggresome formation by a cytosolic GFP-chimera.Journal of Cell Biology.  146:1239-1254. 1999
    1998 The mechanism underlying cystic fibrosis transmembrane conductance regulator transport from the endoplasmic reticulum to the proteasome includes Sec61beta and a cytosolic, deglycosylated intermediary.Journal of Biological Chemistry.  273:29873-29878. 1998
    1998 Cystic fibrosis transmembrane conductance regulator (CFTR) nucleotide-binding domain 1 (NBD-1) and CFTR truncated within NBD-1 target to the epithelial plasma membrane and increase anion permeability.Biochemistry.  37:15222-15230. 1998
    1998 Activation of DeltaF508 CFTR in an epithelial monolayer.American Journal of Physiology.  275:C599-C607. 1998
    1997 Suppression of a CFTR premature stop mutation in a bronchial epithelial cell line.Nature Medicine.  3:1280-1284. 1997
    1997 Purification, characterization, and expression of CFTR nucleotide-binding domains.Journal of Bioenergetics and Biomembranes.  29:475-482. 1997
    1996 Efficiency of plasmid delivery and expression after lipid-mediated gene transfer to human cells in vitro.Journal of Pharmacology and Experimental Therapeutics.  279:1462-1469. 1996
    1996 A method for the rapid detection of recombinant CFTR during gene therapy in cystic fibrosis.Gene Therapy.  3:685-694. 1996
    1996 Development of drug targeting based on recombinant expression of the chicken avidin gene.Journal of Drug Targeting.  4:41-49. 1996
    1995 Bystander killing of melanoma cells using the human tyrosinase promoter to express the Escherichia coli purine nucleoside phosphorylase gene.Cancer Research.  55:3339-3345. 1995
    1995 Severe phenotype in mice with termination mutation in exon 2 of cystic fibrosis gene.Somatic Cell and Molecular Genetics.  21:177-187. 1995
    1995 Cationic lipids for reporter gene and CFTR transfer to rat pulmonary epithelium.Gene Therapy.  2:38-49. 1995
    1995 Cationic lipids for reporter gene and CFTR transfer to rat pulmonary epithelium.Gene Therapy.  2:38-49. 1995
    1994 Informed consent to participate in a research studyHuman Gene Therapy.  5:1271-1277. 1994
    1994 Gene therapy for cystic fibrosis using cationic liposome mediated gene transfer: a phase I trial of safety and efficacy in the nasal airway.Human Gene Therapy.  5:1259-1277. 1994
    1994 Informed consent to participate in a research study -- gene therapy for cystic fibrosis using cationic liposome mediated gene transfer: a phase I trial of safety and efficacy in the nasal airway.Human Gene Therapy.  5:1271-1277. 1994
    1994 Tumor cell bystander killing in colonic carcinoma utilizing the Escherichia coli DeoD gene to generate toxic purines.Gene Therapy.  1:233-238. 1994
    1994 Prognostic relevance of transforming growth factor alpha (TGF-alpha) and tumor necrosis factor alpha (TNF-alpha) detected in breast cancer tissues by immunohistochemistry.Breast Cancer Research and Treatment.  29:229-235. 1994
    1993 [Creation of monoclonal antibodies against tumor necrosis factor-alpha (TNF-alpha) and transforming growth factor alpha (TFG-alpha), their definition and possible use].Orvosi Hetilap.  134:1303-1307. 1993
    1992 Cellular enzyme-linked immunocircle assay. A rapid assay of hybridomas produced against cell surface antigens.Journal of Immunological Methods.  153:141-149. 1992
    1991 Long-term effect of immunization with neural and nonneural antigens on the noradrenaline and serotonin levels of discrete brain areas in mice and the relationship between neurotransmitter levels and antibody production.Acta Physiologica Hungarica.  77:85-92. 1991
    1988 Oligopeptides and phagocytosis in mice.Acta paediatrica Hungarica.  29:45-48. 1988

    Book

    Year Title Altmetric
    2008 Chapter 1 Structure and Functions of Biomembranes.  Ed. 61.  2008

    Education And Training

  • University of Alabama at Birmingham, Birmingham Physiology and Biophysics, Postdoctoral Research
  • Doctor of Medicine, Foreign University
  • Haynal Imre University of Health Sciences, Residency
  • Full Name

  • Zsuzsanna Bebok
  • Blazerid

  • bebok