University of Pittsburgh Department of Cell Biology
    • Raymond A. Frizzell, Ph.D.

    Raymond A. Frizzell, Ph.D.

    Professor, Director of Cystic Fibrosis Research Center
    Dept of Pediatrics, Children's Hospital
    Tel: 412-692-9449
    Fax: 412-692-8906
    Address: 7116 Rangos Research Center, Children's Hospital of Pittsburgh
  • Research

    Our group is interested in the mechanisms of chloride and sodium transport across absorptive and secretory epithelial cells of the lung and kidney, in the biogenesis and quality control pathways that influence ion channel expression, and in the membrane trafficking mechanisms that govern channel density at the cell surface. This work focuses primarily on the anion channel, CFTR, whose mutation produces the genetic disease, cystic fibrosis, and on the epithelial sodium channel, ENaC, a determinant of airway surface liquid volume and blood pressure regulation.

  • Publications

    1. Ahner A, Gong X, Frizzell RA. Cystic fibrosis transmembrane conductance regulator degradation: cross-talk between the ubiquitylation and SUMOylation pathways. FEBS J. 2013 Sep;280(18):4430-8. doi: 10.1111/febs.12415. Epub 2013 Jul 22. Review. PubMed PMID: 23809253.
    2. Holleran JP, Zeng J, Frizzell RA, Watkins SC. Regulated recycling of mutant CFTR is partially restored by pharmacological treatment. J Cell Sci. 2013 Jun 15;126(Pt 12):2692-703. doi: 10.1242/jcs.120196. Epub 2013 Apr 9. PubMed PMID: 23572510; PubMed Central PMCID: PMC3687701.
    3. Ahner A, Gong X, Schmidt BZ, Peters KW, Rabeh WM, Thibodeau PH, Lukacs GL, Frizzell RA. Small heat shock proteins target mutant cystic fibrosis transmembrane conductance regulator for degradation via a small ubiquitin-like modifier-dependent pathway. Mol Biol Cell. 2013 Jan;24(2):74-84. doi: 10.1091/mbc.E12-09-0678. Epub 2012 Nov 14. PubMed PMID: 23155000; PubMed Central PMCID: PMC3541966.
    4. Frizzell RA, Hanrahan JW. Physiology of epithelial chloride and fluid secretion. Cold Spring Harb Perspect Med. 2012 Jun;2(6):a009563. doi: 10.1101/cshperspect.a009563. PubMed PMID: 22675668; PubMed Central PMCID: PMC3367533.
    5. Holleran JP, Glover ML, Peters KW, Bertrand CA, Watkins SC, Jarvik JW, Frizzell RA. Pharmacological rescue of the mutant cystic fibrosis transmembrane conductance regulator (CFTR) detected by use of a novel fluorescence platform. Mol Med. 2012 May 9;18:685-96. doi: 10.2119/molmed.2012.00001. PubMed PMID: 22396015; PubMed Central PMCID: PMC3388127.
    6. Liang X, Da Paula AC, Bozóky Z, Zhang H, Bertrand CA, Peters KW, Forman-Kay JD, Frizzell RA. Phosphorylation-dependent 14-3-3 protein interactions regulate CFTR biogenesis. Mol Biol Cell. 2012 Mar;23(6):996-1009. doi: 10.1091/mbc.E11-08-0662. Epub 2012 Jan 25. PubMed PMID: 22278744; PubMed Central PMCID: PMC3302758.
    7. Butterworth MB, Edinger RS, Silvis MR, Gallo LI, Liang X, Apodaca G, Frizzell RA, Johnson JP. Rab11b regulates the trafficking and recycling of the epithelial sodium channel (ENaC). Am J Physiol Renal Physiol. 2012 Mar 1;302(5):F581-90. doi: 10.​1152/​ajprenal.​00304.​2011. Epub 2011 Nov 30. Erratum in: Am J Physiol Renal Physiol. 2012 Jul;303(1):F163. Fizzell, Raymond A [corrected to Frizzell, Raymond A]. PubMed PMID: 22129970; PubMed Central PMCID: PMC3353647.
    8. Van Goor F, Hadida S, Grootenhuis PD, Burton B, Stack JH, Straley KS, Decker CJ, Miller M, McCartney J, Olson ER, Wine JJ, Frizzell RA, Ashlock M, Negulescu PA. Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809. Proc Natl Acad Sci U S A. 2011 Nov 15;108(46):18843-8. doi: 10.1073/pnas.1105787108. Epub 2011 Oct 5. PubMed PMID: 21976485; PubMed Central PMCID: PMC3219147.
    9. Peters KW, Okiyoneda T, Balch WE, Braakman I, Brodsky JL, Guggino WB, Penland CM, Pollard HB, Sorscher EJ, Skach WR, Thomas PJ, Lukacs GL, Frizzell RA. CFTR Folding Consortium: methods available for studies of CFTR folding and correction. Methods Mol Biol. 2011;742:335-53. doi: 10.1007/978-1-61779-120-8_20. PubMed PMID: 21547742; PubMed Central PMCID: PMC3266683.
    10. Liang X, Butterworth MB, Peters KW, Frizzell RA. AS160 modulates aldosterone-stimulated epithelial sodium channel forward trafficking. Mol Biol Cell. 2010 Jun 15;21(12):2024-33. doi: 10.1091/mbc.E10-01-0042. Epub 2010 Apr 21. PubMed PMID: 20410134; PubMed Central PMCID: PMC2883946.


Quick Links