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Items: 47

1.

Thymosin α-1 does not correct F508del-CFTR in cystic fibrosis airway epithelia.

Tomati V, Caci E, Ferrera L, Pesce E, Sondo E, Cholon DM, Quinney NL, Boyles SE, Armirotti A, Ravazzolo R, Galietta LJ, Gentzsch M, Pedemonte N.

JCI Insight. 2018 Feb 8;3(3). pii: 98699. doi: 10.1172/jci.insight.98699. [Epub ahead of print]

2.

Nasospheroids permit measurements of CFTR-dependent fluid transport.

Guimbellot JS, Leach JM, Chaudhry IG, Quinney NL, Boyles SE, Chua M, Aban I, Jaspers I, Gentzsch M.

JCI Insight. 2017 Nov 16;2(22). pii: 95734. doi: 10.1172/jci.insight.95734. [Epub ahead of print]

3.

Recent progress in translational cystic fibrosis research using precision medicine strategies.

Cholon DM, Gentzsch M.

J Cyst Fibros. 2018 Mar;17(2S):S52-S60. doi: 10.1016/j.jcf.2017.09.005. Epub 2017 Oct 4. Review.

PMID:
28986017
4.

Pharmacological Rescue of Conditionally Reprogrammed Cystic Fibrosis Bronchial Epithelial Cells.

Gentzsch M, Boyles SE, Cheluvaraju C, Chaudhry IG, Quinney NL, Cho C, Dang H, Liu X, Schlegel R, Randell SH.

Am J Respir Cell Mol Biol. 2017 May;56(5):568-574. doi: 10.1165/rcmb.2016-0276MA.

PMID:
27983869
5.

Efficacy of lumacaftor-ivacaftor for the treatment of cystic fibrosis patients homozygous for the F508del-CFTR mutation.

Cholon DM, Esther CR Jr, Gentzsch M.

Expert Rev Precis Med Drug Dev. 2016;1(3):235-243. Epub 2016 Apr 22.

6.

Restoration of R117H CFTR folding and function in human airway cells through combination treatment with VX-809 and VX-770.

Gentzsch M, Ren HY, Houck SA, Quinney NL, Cholon DM, Sopha P, Chaudhry IG, Das J, Dokholyan NV, Randell SH, Cyr DM.

Am J Physiol Lung Cell Mol Physiol. 2016 Sep 1;311(3):L550-9. doi: 10.1152/ajplung.00186.2016. Epub 2016 Jul 8.

7.

The Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Uses its C-Terminus to Regulate the A2B Adenosine Receptor.

Watson MJ, Lee SL, Marklew AJ, Gilmore RC, Gentzsch M, Sassano MF, Gray MA, Tarran R.

Sci Rep. 2016 Jun 9;6:27390. doi: 10.1038/srep27390.

8.

Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis.

Cholon DM, Quinney NL, Fulcher ML, Esther CR Jr, Das J, Dokholyan NV, Randell SH, Boucher RC, Gentzsch M.

Sci Transl Med. 2014 Jul 23;6(246):246ra96. doi: 10.1126/scitranslmed.3008680.

9.

The N-terminal domain allosterically regulates cleavage and activation of the epithelial sodium channel.

Kota P, Buchner G, Chakraborty H, Dang YL, He H, Garcia GJ, Kubelka J, Gentzsch M, Stutts MJ, Dokholyan NV.

J Biol Chem. 2014 Aug 15;289(33):23029-42. doi: 10.1074/jbc.M114.570952. Epub 2014 Jun 28.

10.

Energetic and structural basis for activation of the epithelial sodium channel by matriptase.

Kota P, García-Caballero A, Dang H, Gentzsch M, Stutts MJ, Dokholyan NV.

Biochemistry. 2012 Apr 24;51(16):3460-9. doi: 10.1021/bi2014773. Epub 2012 Apr 9.

11.

Allosteric modulation balances thermodynamic stability and restores function of ΔF508 CFTR.

Aleksandrov AA, Kota P, Cui L, Jensen T, Alekseev AE, Reyes S, He L, Gentzsch M, Aleksandrov LA, Dokholyan NV, Riordan JR.

J Mol Biol. 2012 May 25;419(1-2):41-60. doi: 10.1016/j.jmb.2012.03.001. Epub 2012 Mar 8.

12.

Cigarette smoke exposure induces CFTR internalization and insolubility, leading to airway surface liquid dehydration.

Clunes LA, Davies CM, Coakley RD, Aleksandrov AA, Henderson AG, Zeman KL, Worthington EN, Gentzsch M, Kreda SM, Cholon D, Bennett WD, Riordan JR, Boucher RC, Tarran R.

FASEB J. 2012 Feb;26(2):533-45. doi: 10.1096/fj.11-192377. Epub 2011 Oct 11.

13.

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;7(5):e1002053. doi: 10.1371/journal.ppat.1002053. Epub 2011 May 19.

14.

Imaging CFTR protein localization in cultured cells and tissues.

Kreda SM, Gentzsch M.

Methods Mol Biol. 2011;742:15-33. doi: 10.1007/978-1-61779-120-8_2.

PMID:
21547724
15.

The cystic fibrosis transmembrane conductance regulator impedes proteolytic stimulation of the epithelial Na+ channel.

Gentzsch M, Dang H, Dang Y, Garcia-Caballero A, Suchindran H, Boucher RC, Stutts MJ.

J Biol Chem. 2010 Oct 15;285(42):32227-32. doi: 10.1074/jbc.M110.155259. Epub 2010 Aug 13.

16.

Regulatory insertion removal restores maturation, stability and function of DeltaF508 CFTR.

Aleksandrov AA, Kota P, Aleksandrov LA, He L, Jensen T, Cui L, Gentzsch M, Dokholyan NV, Riordan JR.

J Mol Biol. 2010 Aug 13;401(2):194-210. doi: 10.1016/j.jmb.2010.06.019. Epub 2010 Jun 16.

17.

Regulation of the epithelial Na+ channel and airway surface liquid volume by serine proteases.

Gaillard EA, Kota P, Gentzsch M, Dokholyan NV, Stutts MJ, Tarran R.

Pflugers Arch. 2010 Jun;460(1):1-17. doi: 10.1007/s00424-010-0827-z. Epub 2010 Apr 18. Review.

18.

Modulation of endocytic trafficking and apical stability of CFTR in primary human airway epithelial cultures.

Cholon DM, O'Neal WK, Randell SH, Riordan JR, Gentzsch M.

Am J Physiol Lung Cell Mol Physiol. 2010 Mar;298(3):L304-14. doi: 10.1152/ajplung.00016.2009. Epub 2009 Dec 11.

19.

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.

20.

Dynasore inhibits removal of wild-type and DeltaF508 cystic fibrosis transmembrane conductance regulator (CFTR) from the plasma membrane.

Young A, Gentzsch M, Abban CY, Jia Y, Meneses PI, Bridges RJ, Bradbury NA.

Biochem J. 2009 Jul 15;421(3):377-85. doi: 10.1042/BJ20090389.

PMID:
19442237
21.

Novel human bronchial epithelial cell lines for cystic fibrosis research.

Fulcher ML, Gabriel SE, Olsen JC, Tatreau JR, Gentzsch M, Livanos E, Saavedra MT, Salmon P, Randell SH.

Am J Physiol Lung Cell Mol Physiol. 2009 Jan;296(1):L82-91. doi: 10.1152/ajplung.90314.2008. Epub 2008 Oct 31.

22.

An expanded biological repertoire for Ins(3,4,5,6)P4 through its modulation of ClC-3 function.

Mitchell J, Wang X, Zhang G, Gentzsch M, Nelson DJ, Shears SB.

Curr Biol. 2008 Oct 28;18(20):1600-5. doi: 10.1016/j.cub.2008.08.073.

23.

Role of N-linked oligosaccharides in the biosynthetic processing of the cystic fibrosis membrane conductance regulator.

Chang XB, Mengos A, Hou YX, Cui L, Jensen TJ, Aleksandrov A, Riordan JR, Gentzsch M.

J Cell Sci. 2008 Sep 1;121(Pt 17):2814-23. doi: 10.1242/jcs.028951. Epub 2008 Aug 5.

24.

Direct interaction with filamins modulates the stability and plasma membrane expression of CFTR.

Thelin WR, Chen Y, Gentzsch M, Kreda SM, Sallee JL, Scarlett CO, Borchers CH, Jacobson K, Stutts MJ, Milgram SL.

J Clin Invest. 2007 Feb;117(2):364-74. Epub 2007 Jan 18.

25.

Misassembled mutant DeltaF508 CFTR in the distal secretory pathway alters cellular lipid trafficking.

Gentzsch M, Choudhury A, Chang XB, Pagano RE, Riordan JR.

J Cell Sci. 2007 Feb 1;120(Pt 3):447-55. Epub 2007 Jan 9.

26.

Domain interdependence in the biosynthetic assembly of CFTR.

Cui L, Aleksandrov L, Chang XB, Hou YX, He L, Hegedus T, Gentzsch M, Aleksandrov A, Balch WE, Riordan JR.

J Mol Biol. 2007 Jan 26;365(4):981-94. Epub 2006 Nov 10.

PMID:
17113596
27.

F508del CFTR with two altered RXR motifs escapes from ER quality control but its channel activity is thermally sensitive.

Hegedus T, Aleksandrov A, Cui L, Gentzsch M, Chang XB, Riordan JR.

Biochim Biophys Acta. 2006 May;1758(5):565-72. Epub 2006 Mar 31.

28.

The role of cystic fibrosis transmembrane conductance regulator phenylalanine 508 side chain in ion channel gating.

Cui L, Aleksandrov L, Hou YX, Gentzsch M, Chen JH, Riordan JR, Aleksandrov AA.

J Physiol. 2006 Apr 15;572(Pt 2):347-58. Epub 2006 Feb 16.

29.

SERCA pump inhibitors do not correct biosynthetic arrest of deltaF508 CFTR in cystic fibrosis.

Grubb BR, Gabriel SE, Mengos A, Gentzsch M, Randell SH, Van Heeckeren AM, Knowles MR, Drumm ML, Riordan JR, Boucher RC.

Am J Respir Cell Mol Biol. 2006 Mar;34(3):355-63. Epub 2005 Nov 11.

30.

Bcr (breakpoint cluster region) protein binds to PDZ-domains of scaffold protein PDZK1 and vesicle coat protein Mint3.

Malmberg EK, Andersson CX, Gentzsch M, Chen JH, Mengos A, Cui L, Hansson GC, Riordan JR.

J Cell Sci. 2004 Nov 1;117(Pt 23):5535-41. Epub 2004 Oct 19.

31.

Biochemical methods to assess CFTR expression and membrane localization.

Farinha CM, Penque D, Roxo-Rosa M, Lukacs G, Dormer R, McPherson M, Pereira M, Bot AG, Jorna H, Willemsen R, Dejonge H, Heda GD, Marino CR, Fanen P, Hinzpeter A, Lipecka J, Fritsch J, Gentzsch M, Edelman A, Amaral MD.

J Cyst Fibros. 2004 Aug;3 Suppl 2:73-7.

32.

Antibodies for CFTR studies.

Mendes F, Farinha CM, Roxo-Rosa M, Fanen P, Edelman A, Dormer R, McPherson M, Davidson H, Puchelle E, De Jonge H, Heda GD, Gentzsch M, Lukacs G, Penque D, Amaral MD.

J Cyst Fibros. 2004 Aug;3 Suppl 2:69-72. Review.

33.

Endocytic trafficking routes of wild type and DeltaF508 cystic fibrosis transmembrane conductance regulator.

Gentzsch M, Chang XB, Cui L, Wu Y, Ozols VV, Choudhury A, Pagano RE, Riordan JR.

Mol Biol Cell. 2004 Jun;15(6):2684-96. Epub 2004 Apr 9.

34.

The PDZ-binding chloride channel ClC-3B localizes to the Golgi and associates with cystic fibrosis transmembrane conductance regulator-interacting PDZ proteins.

Gentzsch M, Cui L, Mengos A, Chang XB, Chen JH, Riordan JR.

J Biol Chem. 2003 Feb 21;278(8):6440-9. Epub 2002 Dec 5.

36.

Expression and degradation of the cystic fibrosis transmembrane conductance regulator in Saccharomyces cerevisiae.

Kiser GL, Gentzsch M, Kloser AK, Balzi E, Wolf DH, Goffeau A, Riordan JR.

Arch Biochem Biophys. 2001 Jun 15;390(2):195-205.

PMID:
11396922
38.

O-Glycosylation of Axl2/Bud10p by Pmt4p is required for its stability, localization, and function in daughter cells.

Sanders SL, Gentzsch M, Tanner W, Herskowitz I.

J Cell Biol. 1999 Jun 14;145(6):1177-88.

39.

Protein O-mannosylation.

Strahl-Bolsinger S, Gentzsch M, Tanner W.

Biochim Biophys Acta. 1999 Jan 6;1426(2):297-307. Review.

PMID:
9878797
41.

Protein-O-glycosylation in yeast: protein-specific mannosyltransferases.

Gentzsch M, Tanner W.

Glycobiology. 1997 Jun;7(4):481-6.

PMID:
9184828
42.
43.
44.
45.
46.

A new Dol-P-Man:protein O-D-mannosyltransferase activity from Saccharomyces cerevisiae.

Gentzsch M, Strahl-Bolsinger S, Tanner W.

Glycobiology. 1995 Feb;5(1):77-82.

PMID:
7772870
47.

Fungal glycoproteins and their biosynthetic pathway as potential targets for antifungal agents.

Tanner W, Gentzsch M, Immervoll T, Scheinost A, Strahl-Bolsinger S.

Acta Biochim Pol. 1995;42(4):505-8. Review.

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