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199 related items for PubMed ID: 30485852

  • 1. Syntaxin 8 and the Endoplasmic Reticulum Processing of ΔF508-CFTR.
    Sabirzhanova I, Boinot C, Guggino WB, Cebotaru L.
    Cell Physiol Biochem; 2018; 51(3):1489-1499. PubMed ID: 30485852
    [Abstract] [Full Text] [Related]

  • 2. 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 01; 287(23):19158-70. PubMed ID: 22505710
    [Abstract] [Full Text] [Related]

  • 3. Control of cystic fibrosis transmembrane conductance regulator membrane trafficking: not just from the endoplasmic reticulum to the Golgi.
    Farinha CM, Matos P, Amaral MD.
    FEBS J; 2013 Sep 01; 280(18):4396-406. PubMed ID: 23773658
    [Abstract] [Full Text] [Related]

  • 4. AAV exploits subcellular stress associated with inflammation, endoplasmic reticulum expansion, and misfolded proteins in models of cystic fibrosis.
    Johnson JS, Gentzsch M, Zhang L, Ribeiro CM, Kantor B, Kafri T, Pickles RJ, Samulski RJ.
    PLoS Pathog; 2011 May 01; 7(5):e1002053. PubMed ID: 21625534
    [Abstract] [Full Text] [Related]

  • 5. The CFTR-Associated Ligand Arrests the Trafficking of the Mutant ΔF508 CFTR Channel in the ER Contributing to Cystic Fibrosis.
    Bergbower E, Boinot C, Sabirzhanova I, Guggino W, Cebotaru L.
    Cell Physiol Biochem; 2018 May 01; 45(2):639-655. PubMed ID: 29402832
    [Abstract] [Full Text] [Related]

  • 6. Ribosomal Stalk Protein Silencing Partially Corrects the ΔF508-CFTR Functional Expression Defect.
    Veit G, Oliver K, Apaja PM, Perdomo D, Bidaud-Meynard A, Lin ST, Guo J, Icyuz M, Sorscher EJ, Hartman JL, Lukacs GL.
    PLoS Biol; 2016 May 01; 14(5):e1002462. PubMed ID: 27168400
    [Abstract] [Full Text] [Related]

  • 7. ERp29 regulates DeltaF508 and wild-type cystic fibrosis transmembrane conductance regulator (CFTR) trafficking to the plasma membrane in cystic fibrosis (CF) and non-CF epithelial cells.
    Suaud L, Miller K, Alvey L, Yan W, Robay A, Kebler C, Kreindler JL, Guttentag S, Hubbard MJ, Rubenstein RC.
    J Biol Chem; 2011 Jun 17; 286(24):21239-53. PubMed ID: 21525008
    [Abstract] [Full Text] [Related]

  • 8. Coupling cystic fibrosis to endoplasmic reticulum stress: Differential role of Grp78 and ATF6.
    Kerbiriou M, Le Drévo MA, Férec C, Trouvé P.
    Biochim Biophys Acta; 2007 Dec 17; 1772(11-12):1236-49. PubMed ID: 18022401
    [Abstract] [Full Text] [Related]

  • 9. Mechanistic Approaches to Improve Correction of the Most Common Disease-Causing Mutation in Cystic Fibrosis.
    Bali V, Lazrak A, Guroji P, Matalon S, Bebok Z.
    PLoS One; 2016 Dec 17; 11(5):e0155882. PubMed ID: 27214033
    [Abstract] [Full Text] [Related]

  • 10. Diffusional mobility of the cystic fibrosis transmembrane conductance regulator mutant, delta F508-CFTR, in the endoplasmic reticulum measured by photobleaching of GFP-CFTR chimeras.
    Haggie PM, Stanton BA, Verkman AS.
    J Biol Chem; 2002 May 10; 277(19):16419-25. PubMed ID: 11877404
    [Abstract] [Full Text] [Related]

  • 11. Role of calnexin in the ER quality control and productive folding of CFTR; differential effect of calnexin knockout on wild-type and DeltaF508 CFTR.
    Okiyoneda T, Niibori A, Harada K, Kohno T, Michalak M, Duszyk M, Wada I, Ikawa M, Shuto T, Suico MA, Kai H.
    Biochim Biophys Acta; 2008 Sep 10; 1783(9):1585-94. PubMed ID: 18457676
    [Abstract] [Full Text] [Related]

  • 12. The DeltaF508 mutation results in loss of CFTR function and mature protein in native human colon.
    Mall M, Kreda SM, Mengos A, Jensen TJ, Hirtz S, Seydewitz HH, Yankaskas J, Kunzelmann K, Riordan JR, Boucher RC.
    Gastroenterology; 2004 Jan 10; 126(1):32-41. PubMed ID: 14699484
    [Abstract] [Full Text] [Related]

  • 13. Selective inhibition of endoplasmic reticulum-associated degradation rescues DeltaF508-cystic fibrosis transmembrane regulator and suppresses interleukin-8 levels: therapeutic implications.
    Vij N, Fang S, Zeitlin PL.
    J Biol Chem; 2006 Jun 23; 281(25):17369-17378. PubMed ID: 16621797
    [Abstract] [Full Text] [Related]

  • 14. Protein processing and inflammatory signaling in Cystic Fibrosis: challenges and therapeutic strategies.
    Belcher CN, Vij N.
    Curr Mol Med; 2010 Feb 23; 10(1):82-94. PubMed ID: 20205681
    [Abstract] [Full Text] [Related]

  • 15. Functional cystic fibrosis transmembrane conductance regulator tagged with an epitope of the vesicular stomatis virus glycoprotein can be addressed to the apical domain of polarized cells.
    Costa de Beauregard MA, Edelman A, Chesnoy-Marchais D, Tondelier D, Lapillonne A, El Marjou F, Robine S, Louvard D.
    Eur J Cell Biol; 2000 Nov 23; 79(11):795-802. PubMed ID: 11139142
    [Abstract] [Full Text] [Related]

  • 16. Processing and function of CFTR-DeltaF508 are species-dependent.
    Ostedgaard LS, Rogers CS, Dong Q, Randak CO, Vermeer DW, Rokhlina T, Karp PH, Welsh MJ.
    Proc Natl Acad Sci U S A; 2007 Sep 25; 104(39):15370-5. PubMed ID: 17873061
    [Abstract] [Full Text] [Related]

  • 17. Syntaxin 6 and CAL mediate the degradation of the cystic fibrosis transmembrane conductance regulator.
    Cheng J, Cebotaru V, Cebotaru L, Guggino WB.
    Mol Biol Cell; 2010 Apr 01; 21(7):1178-87. PubMed ID: 20130090
    [Abstract] [Full Text] [Related]

  • 18. Ubiquitination and degradation of CFTR by the E3 ubiquitin ligase MARCH2 through its association with adaptor proteins CAL and STX6.
    Cheng J, Guggino W.
    PLoS One; 2013 Apr 01; 8(6):e68001. PubMed ID: 23818989
    [Abstract] [Full Text] [Related]

  • 19. Endogenous surface expression of ΔF508-CFTR mediates cAMP-stimulated Cl(-) current in CFTR(ΔF508/ΔF508) pig thyroid epithelial cells.
    Li Y, Ganta S, Fong P.
    Exp Physiol; 2012 Jan 01; 97(1):115-24. PubMed ID: 21948195
    [Abstract] [Full Text] [Related]

  • 20. Rescuing cystic fibrosis transmembrane conductance regulator (CFTR)-processing mutants by transcomplementation.
    Cormet-Boyaka E, Jablonsky M, Naren AP, Jackson PL, Muccio DD, Kirk KL.
    Proc Natl Acad Sci U S A; 2004 May 25; 101(21):8221-6. PubMed ID: 15141088
    [Abstract] [Full Text] [Related]

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