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Items: 1 to 50 of 199

1.

Different SUMO paralogues determine the fate of wild-type and mutant CFTRs: biogenesis versus degradation.

Gong X, Liao Y, Ahner A, Larsen MB, Wang X, Bertrand CA, Frizzell RA.

Mol Biol Cell. 2019 Jan 1;30(1):4-16. doi: 10.1091/mbc.E18-04-0252. Epub 2018 Nov 7.

2.

Silencing of the Hsp70-specific nucleotide-exchange factor BAG3 corrects the F508del-CFTR variant by restoring autophagy.

Hutt DM, Mishra SK, Roth DM, Larsen MB, Angles F, Frizzell RA, Balch WE.

J Biol Chem. 2018 Aug 31;293(35):13682-13695. doi: 10.1074/jbc.RA118.002607. Epub 2018 Jul 9.

PMID:
29986884
3.

The CFTR trafficking mutation F508del inhibits the constitutive activity of SLC26A9.

Bertrand CA, Mitra S, Mishra SK, Wang X, Zhao Y, Pilewski JM, Madden DR, Frizzell RA.

Am J Physiol Lung Cell Mol Physiol. 2017 Jun 1;312(6):L912-L925. doi: 10.1152/ajplung.00178.2016. Epub 2017 Mar 30.

4.

Functional and molecular identification of a TASK-1 potassium channel regulating chloride secretion through CFTR channels in the shark rectal gland: implications for cystic fibrosis.

Telles CJ, Decker SE, Motley WW, Peters AW, Mehr AP, Frizzell RA, Forrest JN Jr.

Am J Physiol Cell Physiol. 2016 Dec 1;311(6):C884-C894. doi: 10.1152/ajpcell.00030.2016. Epub 2016 Sep 21.

5.

From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations.

Veit G, Avramescu RG, Chiang AN, Houck SA, Cai Z, Peters KW, Hong JS, Pollard HB, Guggino WB, Balch WE, Skach WR, Cutting GR, Frizzell RA, Sheppard DN, Cyr DM, Sorscher EJ, Brodsky JL, Lukacs GL.

Mol Biol Cell. 2016 Feb 1;27(3):424-33. doi: 10.1091/mbc.E14-04-0935.

6.

VAMP-associated Proteins (VAP) as Receptors That Couple Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Proteostasis with Lipid Homeostasis.

Ernst WL, Shome K, Wu CC, Gong X, Frizzell RA, Aridor M.

J Biol Chem. 2016 Mar 4;291(10):5206-20. doi: 10.1074/jbc.M115.692749. Epub 2016 Jan 6.

7.

Non-native Conformers of Cystic Fibrosis Transmembrane Conductance Regulator NBD1 Are Recognized by Hsp27 and Conjugated to SUMO-2 for Degradation.

Gong X, Ahner A, Roldan A, Lukacs GL, Thibodeau PH, Frizzell RA.

J Biol Chem. 2016 Jan 22;291(4):2004-17. doi: 10.1074/jbc.M115.685628. Epub 2015 Dec 1.

8.

Divergent signaling via SUMO modification: potential for CFTR modulation.

Ahner A, Gong X, Frizzell RA.

Am J Physiol Cell Physiol. 2016 Feb 1;310(3):C175-80. doi: 10.1152/ajpcell.00124.2015. Epub 2015 Nov 18. Review.

9.

A combination therapy for cystic fibrosis.

Brodsky JL, Frizzell RA.

Cell. 2015 Sep 24;163(1):17. doi: 10.1016/j.cell.2015.09.003.

10.

Simple image-based no-wash method for quantitative detection of surface expressed CFTR.

Larsen MB, Hu J, Frizzell RA, Watkins SC.

Methods. 2016 Mar 1;96:40-45. doi: 10.1016/j.ymeth.2015.09.006. Epub 2015 Sep 7.

11.

miR-16 rescues F508del-CFTR function in native cystic fibrosis epithelial cells.

Kumar P, Bhattacharyya S, Peters KW, Glover ML, Sen A, Cox RT, Kundu S, Caohuy H, Frizzell RA, Pollard HB, Biswas R.

Gene Ther. 2015 Nov;22(11):908-16. doi: 10.1038/gt.2015.56. Epub 2015 Jul 2.

12.

SUMOylation Modulates CFTR Biogenesis: Is the Pathway Druggable?

Ahner A, Frizzell RA.

Curr Drug Targets. 2015;16(9):965-75. Review.

PMID:
26028049
13.
14.

Regulatory R region of the CFTR chloride channel is a dynamic integrator of phospho-dependent intra- and intermolecular interactions.

Bozoky Z, Krzeminski M, Muhandiram R, Birtley JR, Al-Zahrani A, Thomas PJ, Frizzell RA, Ford RC, Forman-Kay JD.

Proc Natl Acad Sci U S A. 2013 Nov 19;110(47):E4427-36. doi: 10.1073/pnas.1315104110. Epub 2013 Nov 4.

15.

Cystic fibrosis transmembrane conductance regulator degradation: cross-talk between the ubiquitylation and SUMOylation pathways.

Ahner A, Gong X, Frizzell RA.

FEBS J. 2013 Sep;280(18):4430-8. doi: 10.1111/febs.12415. Epub 2013 Jul 22. Review.

16.

Regulated recycling of mutant CFTR is partially restored by pharmacological treatment.

Holleran JP, Zeng J, Frizzell RA, Watkins SC.

J Cell Sci. 2013 Jun 15;126(Pt 12):2692-703. doi: 10.1242/jcs.120196. Epub 2013 Apr 9.

17.

Small heat shock proteins target mutant cystic fibrosis transmembrane conductance regulator for degradation via a small ubiquitin-like modifier-dependent pathway.

Ahner A, Gong X, Schmidt BZ, Peters KW, Rabeh WM, Thibodeau PH, Lukacs GL, Frizzell RA.

Mol Biol Cell. 2013 Jan;24(2):74-84. doi: 10.1091/mbc.E12-09-0678. Epub 2012 Nov 14.

18.

Physiology of epithelial chloride and fluid secretion.

Frizzell RA, Hanrahan JW.

Cold Spring Harb Perspect Med. 2012 Jun;2(6):a009563. doi: 10.1101/cshperspect.a009563.

19.

Human heat shock protein 105/110 kDa (Hsp105/110) regulates biogenesis and quality control of misfolded cystic fibrosis transmembrane conductance regulator at multiple levels.

Saxena A, Banasavadi-Siddegowda YK, Fan Y, Bhattacharya S, Roy G, Giovannucci DR, Frizzell RA, Wang X.

J Biol Chem. 2012 Jun 1;287(23):19158-70. doi: 10.1074/jbc.M111.297580. Epub 2012 Apr 13.

20.

Pharmacological rescue of the mutant cystic fibrosis transmembrane conductance regulator (CFTR) detected by use of a novel fluorescence platform.

Holleran JP, Glover ML, Peters KW, Bertrand CA, Watkins SC, Jarvik JW, Frizzell RA.

Mol Med. 2012 May 9;18:685-96. doi: 10.2119/molmed.2012.00001.

21.

Phosphorylation-dependent 14-3-3 protein interactions regulate CFTR biogenesis.

Liang X, Da Paula AC, Bozóky Z, Zhang H, Bertrand CA, Peters KW, Forman-Kay JD, Frizzell RA.

Mol Biol Cell. 2012 Mar;23(6):996-1009. doi: 10.1091/mbc.E11-08-0662. Epub 2012 Jan 25.

22.

Rab11b regulates the trafficking and recycling of the epithelial sodium channel (ENaC).

Butterworth MB, Edinger RS, Silvis MR, Gallo LI, Liang X, Apodaca G, Frizzell RA, Johnson JP.

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].

23.

Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809.

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.

Proc Natl Acad Sci U S A. 2011 Nov 15;108(46):18843-8. doi: 10.1073/pnas.1105787108. Epub 2011 Oct 5.

24.

CFTR Folding Consortium: methods available for studies of CFTR folding and correction.

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.

Methods Mol Biol. 2011;742:335-53. doi: 10.1007/978-1-61779-120-8_20.

25.

AS160 modulates aldosterone-stimulated epithelial sodium channel forward trafficking.

Liang X, Butterworth MB, Peters KW, Frizzell RA.

Mol Biol Cell. 2010 Jun 15;21(12):2024-33. doi: 10.1091/mbc.E10-01-0042. Epub 2010 Apr 21.

26.

Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis.

Hutt DM, Herman D, Rodrigues AP, Noel S, Pilewski JM, Matteson J, Hoch B, Kellner W, Kelly JW, Schmidt A, Thomas PJ, Matsumura Y, Skach WR, Gentzsch M, Riordan JR, Sorscher EJ, Okiyoneda T, Yates JR 3rd, Lukacs GL, Frizzell RA, Manning G, Gottesfeld JM, Balch WE.

Nat Chem Biol. 2010 Jan;6(1):25-33. doi: 10.1038/nchembio.275. Epub 2009 Dec 6.

27.

Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770.

Van Goor F, Hadida S, Grootenhuis PD, Burton B, Cao D, Neuberger T, Turnbull A, Singh A, Joubran J, Hazlewood A, Zhou J, McCartney J, Arumugam V, Decker C, Yang J, Young C, Olson ER, Wine JJ, Frizzell RA, Ashlock M, Negulescu P.

Proc Natl Acad Sci U S A. 2009 Nov 3;106(44):18825-30. doi: 10.1073/pnas.0904709106. Epub 2009 Oct 21.

28.

SLC26A9 is a constitutively active, CFTR-regulated anion conductance in human bronchial epithelia.

Bertrand CA, Zhang R, Pilewski JM, Frizzell RA.

J Gen Physiol. 2009 Apr;133(4):421-38. doi: 10.1085/jgp.200810097. Epub 2009 Mar 16.

29.

Rab11b regulates the apical recycling of the cystic fibrosis transmembrane conductance regulator in polarized intestinal epithelial cells.

Silvis MR, Bertrand CA, Ameen N, Golin-Bisello F, Butterworth MB, Frizzell RA, Bradbury NA.

Mol Biol Cell. 2009 Apr;20(8):2337-50. doi: 10.1091/mbc.E08-01-0084. Epub 2009 Feb 25.

31.

Interleukin-17A induces bicarbonate secretion in normal human bronchial epithelial cells.

Kreindler JL, Bertrand CA, Lee RJ, Karasic T, Aujla S, Pilewski JM, Frizzell RA, Kolls JK.

Am J Physiol Lung Cell Mol Physiol. 2009 Feb;296(2):L257-66. doi: 10.1152/ajplung.00344.2007. Epub 2008 Dec 12.

32.

Regulation of CFTR trafficking by its R domain.

Lewarchik CM, Peters KW, Qi J, Frizzell RA.

J Biol Chem. 2008 Oct 17;283(42):28401-12. doi: 10.1074/jbc.M800516200. Epub 2008 Aug 11.

33.

An obligatory heterodimer of 14-3-3beta and 14-3-3epsilon is required for aldosterone regulation of the epithelial sodium channel.

Liang X, Butterworth MB, Peters KW, Walker WH, Frizzell RA.

J Biol Chem. 2008 Oct 10;283(41):27418-25. doi: 10.1074/jbc.M803687200. Epub 2008 Aug 7.

34.

Chaperone displacement from mutant cystic fibrosis transmembrane conductance regulator restores its function in human airway epithelia.

Sun F, Mi Z, Condliffe SB, Bertrand CA, Gong X, Lu X, Zhang R, Latoche JD, Pilewski JM, Robbins PD, Frizzell RA.

FASEB J. 2008 Sep;22(9):3255-63. doi: 10.1096/fj.07-105338. Epub 2008 Jun 12.

35.

Regulation of the epithelial sodium channel by membrane trafficking.

Butterworth MB, Edinger RS, Frizzell RA, Johnson JP.

Am J Physiol Renal Physiol. 2009 Jan;296(1):F10-24. doi: 10.1152/ajprenal.90248.2008. Epub 2008 May 28. Review.

36.

Prostasin expression is regulated by airway surface liquid volume and is increased in cystic fibrosis.

Myerburg MM, McKenna EE, Luke CJ, Frizzell RA, Kleyman TR, Pilewski JM.

Am J Physiol Lung Cell Mol Physiol. 2008 May;294(5):L932-41. doi: 10.1152/ajplung.00437.2007. Epub 2008 Feb 29.

37.

The deubiquitinating enzyme UCH-L3 regulates the apical membrane recycling of the epithelial sodium channel.

Butterworth MB, Edinger RS, Ovaa H, Burg D, Johnson JP, Frizzell RA.

J Biol Chem. 2007 Dec 28;282(52):37885-93. Epub 2007 Oct 29.

38.

The epithelial sodium channel (ENaC) traffics to apical membrane in lipid rafts in mouse cortical collecting duct cells.

Hill WG, Butterworth MB, Wang H, Edinger RS, Lebowitz J, Peters KW, Frizzell RA, Johnson JP.

J Biol Chem. 2007 Dec 28;282(52):37402-11. Epub 2007 Oct 10.

39.

Regulation of human cystic fibrosis transmembrane conductance regulator (CFTR) by serum- and glucocorticoid-inducible kinase (SGK1).

Sato JD, Chapline MC, Thibodeau R, Frizzell RA, Stanton BA.

Cell Physiol Biochem. 2007;20(1-4):91-8.

40.

Electrogenic bicarbonate secretion by prairie dog gallbladder.

Moser AJ, Gangopadhyay A, Bradbury NA, Peters KW, Frizzell RA, Bridges RJ.

Am J Physiol Gastrointest Liver Physiol. 2007 Jun;292(6):G1683-94. Epub 2007 Mar 15.

41.

Small heat-shock proteins select deltaF508-CFTR for endoplasmic reticulum-associated degradation.

Ahner A, Nakatsukasa K, Zhang H, Frizzell RA, Brodsky JL.

Mol Biol Cell. 2007 Mar;18(3):806-14. Epub 2006 Dec 20.

42.

Derlin-1 promotes the efficient degradation of the cystic fibrosis transmembrane conductance regulator (CFTR) and CFTR folding mutants.

Sun F, Zhang R, Gong X, Geng X, Drain PF, Frizzell RA.

J Biol Chem. 2006 Dec 1;281(48):36856-63. Epub 2006 Sep 5.

43.

Methods for detecting internalized, FM 1-43 stained particles in epithelial cells and monolayers.

Bertrand CA, Laboisse C, Hopfer U, Bridges RJ, Frizzell RA.

Biophys J. 2006 Nov 15;91(10):3872-83. Epub 2006 Aug 25.

44.

Airway surface liquid volume regulates ENaC by altering the serine protease-protease inhibitor balance: a mechanism for sodium hyperabsorption in cystic fibrosis.

Myerburg MM, Butterworth MB, McKenna EE, Peters KW, Frizzell RA, Kleyman TR, Pilewski JM.

J Biol Chem. 2006 Sep 22;281(38):27942-9. Epub 2006 Jul 26.

45.

Identification and membrane localization of electrogenic sodium bicarbonate cotransporters in Calu-3 cells.

Kreindler JL, Peters KW, Frizzell RA, Bridges RJ.

Biochim Biophys Acta. 2006 Jul;1762(7):704-10. Epub 2006 Jun 17.

46.

14-3-3 isoforms are induced by aldosterone and participate in its regulation of epithelial sodium channels.

Liang X, Peters KW, Butterworth MB, Frizzell RA.

J Biol Chem. 2006 Jun 16;281(24):16323-32. Epub 2006 Apr 12.

47.

Clathrin-mediated endocytosis of the epithelial sodium channel. Role of epsin.

Wang H, Traub LM, Weixel KM, Hawryluk MJ, Shah N, Edinger RS, Perry CJ, Kester L, Butterworth MB, Peters KW, Kleyman TR, Frizzell RA, Johnson JP.

J Biol Chem. 2006 May 19;281(20):14129-35. Epub 2006 Mar 30.

48.

Cysteine string protein monitors late steps in cystic fibrosis transmembrane conductance regulator biogenesis.

Zhang H, Schmidt BZ, Sun F, Condliffe SB, Butterworth MB, Youker RT, Brodsky JL, Aridor M, Frizzell RA.

J Biol Chem. 2006 Apr 21;281(16):11312-21. Epub 2006 Feb 9.

49.

PKA-dependent ENaC trafficking requires the SNARE-binding protein complexin.

Butterworth MB, Frizzell RA, Johnson JP, Peters KW, Edinger RS.

Am J Physiol Renal Physiol. 2005 Nov;289(5):F969-77. Epub 2005 Jun 21.

50.

Acute ENaC stimulation by cAMP in a kidney cell line is mediated by exocytic insertion from a recycling channel pool.

Butterworth MB, Edinger RS, Johnson JP, Frizzell RA.

J Gen Physiol. 2005 Jan;125(1):81-101.

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