University of Pittsburgh Department of Cell Biology

  • 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. 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. 2011 Nov 30. [Epub ahead of print]
    2. 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.
    3. 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.
    4. 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.
    5. Hutt DM, D. Herman, A.P. Rodrigues, S. Noel, J.M. Pilewski, J. Matteson, B. Hoch, W. Kellner, J.W. Kelly, A. Schmidt, P.J. Thomas, Y. Matsumura, W.R. Skach, M. Gentzsch, J.R. Riordan, E.J. Sorscher, T. Okiyoneda, J.R. Yates 3rd, G.L. Lukacs, R.A. Frizzell, G. Mannin, J.M. Gottesfeld, W.E. Balch. Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis. Nat Chem Biol. 2010 Jan;6(1):25-33.
    6. Butterworth, M.B., R.S. Edinger, R.A. Frizzell, J.P. Johnson. Regulation of epithelial sodium channel (ENaC) by membrane trafficking. Am J Physiol Renal Physiol. 2009 Jan;296(1):F10-24.
    7. Silvis, M.R., C.A. Bertrand, N. Ameen, F. Golin-Bisello, M.B. Butterworth, R.A. Frizzell, N.A. Bradbury. Rab11b Regulates the Apical Recycling of CFTR in Polarized Intestinal Epithelial Cells. Mol Biol Cell. 2009 Apr;20(8):2377-50.
    8. Bertrand, C.A., R. Zhang, J.M. Pilewski, R.A. Frizzell. SLC26A9 is a constitutively active, CFTR-regulated anion conductance in human bronchial epithelia. J Gen Physiol. 2009 Apr; 133(4):421-38.
    9. VanGoor F., S. Hadida, P.D. Grootenhuis, B. Burton, D. Cao, T. Neuberger, A. Turnbull, A. Singh, J. Joubran, A. Hazlewood, J. Zhou, J. McCartney, V. Arumugam, C. Decker, J. Yang, C. Young, E.R. Olson, J.J. Wine, R.A. Frizzell, M. Ashlock, P. Negulescu. Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770. Proc Natl Acad Sci USA, 2009 Nov 3;106(44):18825-30.
    10. Sun F., Z. Mi, S.B. Condliffe, C.A. Bertrand, X. Gong, X. Lu, R. Zhang, J.D. Latoche, J.M. Pilewski, P.D. Robbins, R.A. Frizzell. Chaperone displacement from mutant cystic fibrosis transmembrane conductance regulator restores its function in human airway epithelia. FasebJ. 2008 Sep;22(9):3255-63.
    11. Liang, X., M.B. Butterworth, K.W. Peters, W.H. Walker, R.A. Frizzell. An obligatory heterodimer of 14-3-3beta and 14-3-3episilon is required for aldosterone regulation of the epithelial sodium channel. J Biol Chem. 2008 Oct 10:283(41):27418-25.
    12. Lewarchik, C.M., K.W. Peters, J. Qi, R.A. Frizzell. Regulation of CFTR trafficking by its R domain. J Biol Chem. 2008 Oct 17;283(42):28401-28412.

     

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