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Fibril-induced glutamine-/asparagine-rich prions recruit stress granule proteins in mammalian cells.

Riemschoss K, Arndt V, Bolognesi B, von Eisenhart-Rothe P, Liu S, Buravlova O, Duernberger Y, Paulsen L, Hornberger A, Hossinger A, Lorenzo-Gotor N, Hogl S, Müller SA, Tartaglia G, Lichtenthaler SF, Vorberg IM.

Life Sci Alliance. 2019 Jul 2;2(4). pii: e201800280. doi: 10.26508/lsa.201800280. Print 2019 Aug.


Editorial overview: Prion disease: From drug discovery tools to clinical trials.

Chiesa R, Vorberg IM.

Curr Opin Pharmacol. 2019 Feb;44:iii-v. doi: 10.1016/j.coph.2019.05.002. No abstract available.


All the Same? The Secret Life of Prion Strains within Their Target Cells.

Vorberg IM.

Viruses. 2019 Apr 9;11(4). pii: E334. doi: 10.3390/v11040334. Review.


Experimental models to study prion disease pathogenesis and identify potential therapeutic compounds.

Vorberg I, Chiesa R.

Curr Opin Pharmacol. 2019 Feb;44:28-38. doi: 10.1016/j.coph.2019.02.002. Epub 2019 Mar 14. Review.


Deregulated Splicing Is a Major Mechanism of RNA-Induced Toxicity in Huntington's Disease.

Schilling J, Broemer M, Atanassov I, Duernberger Y, Vorberg I, Dieterich C, Dagane A, Dittmar G, Wanker E, van Roon-Mom W, Winter J, Krauß S.

J Mol Biol. 2019 Apr 19;431(9):1869-1877. doi: 10.1016/j.jmb.2019.01.034. Epub 2019 Jan 31.


Prion Replication in the Mammalian Cytosol: Functional Regions within a Prion Domain Driving Induction, Propagation, and Inheritance.

Duernberger Y, Liu S, Riemschoss K, Paulsen L, Bester R, Kuhn PH, Schölling M, Lichtenthaler SF, Vorberg I.

Mol Cell Biol. 2018 Jul 16;38(15). pii: e00111-18. doi: 10.1128/MCB.00111-18. Print 2018 Aug 1.


Endoplasmic Reticulum Stress Induces Myostatin High Molecular Weight Aggregates and Impairs Mature Myostatin Secretion.

Sachdev R, Kappes-Horn K, Paulsen L, Duernberger Y, Pleschka C, Denner P, Kundu B, Reimann J, Vorberg I.

Mol Neurobiol. 2018 Nov;55(11):8355-8373. doi: 10.1007/s12035-018-0997-9. Epub 2018 Mar 15.


Inhibition of the MID1 protein complex: a novel approach targeting APP protein synthesis.

Matthes F, Hettich MM, Schilling J, Flores-Dominguez D, Blank N, Wiglenda T, Buntru A, Wolf H, Weber S, Vorberg I, Dagane A, Dittmar G, Wanker E, Ehninger D, Krauss S.

Cell Death Discov. 2018 Jan 29;4:4. doi: 10.1038/s41420-017-0003-8. eCollection 2018 Dec.


Mammalian amyloidogenic proteins promote prion nucleation in yeast.

Chandramowlishwaran P, Sun M, Casey KL, Romanyuk AV, Grizel AV, Sopova JV, Rubel AA, Nussbaum-Krammer C, Vorberg IM, Chernoff YO.

J Biol Chem. 2018 Mar 2;293(9):3436-3450. doi: 10.1074/jbc.M117.809004. Epub 2018 Jan 12.


YKL-40 in the brain and cerebrospinal fluid of neurodegenerative dementias.

Llorens F, Thüne K, Tahir W, Kanata E, Diaz-Lucena D, Xanthopoulos K, Kovatsi E, Pleschka C, Garcia-Esparcia P, Schmitz M, Ozbay D, Correia S, Correia Â, Milosevic I, Andréoletti O, Fernández-Borges N, Vorberg IM, Glatzel M, Sklaviadis T, Torres JM, Krasemann S, Sánchez-Valle R, Ferrer I, Zerr I.

Mol Neurodegener. 2017 Nov 10;12(1):83. doi: 10.1186/s13024-017-0226-4.


Genetic human prion disease modelled in PrP transgenic Drosophila.

Thackray AM, Cardova A, Wolf H, Pradl L, Vorberg I, Jackson WS, Bujdoso R.

Biochem J. 2017 Sep 20;474(19):3253-3267. doi: 10.1042/BCJ20170462.


Prion strains depend on different endocytic routes for productive infection.

Fehlinger A, Wolf H, Hossinger A, Duernberger Y, Pleschka C, Riemschoss K, Liu S, Bester R, Paulsen L, Priola SA, Groschup MH, Schätzl HM, Vorberg IM.

Sci Rep. 2017 Jul 31;7(1):6923. doi: 10.1038/s41598-017-07260-2.


Prions on the run: How extracellular vesicles serve as delivery vehicles for self-templating protein aggregates.

Liu S, Hossinger A, Göbbels S, Vorberg IM.

Prion. 2017 Mar 4;11(2):98-112. doi: 10.1080/19336896.2017.1306162. Review.


Horizontal Transmission of Cytosolic Sup35 Prions by Extracellular Vesicles.

Liu S, Hossinger A, Hofmann JP, Denner P, Vorberg IM.

MBio. 2016 Jul 12;7(4). pii: e00915-16. doi: 10.1128/mBio.00915-16.


Deposition pattern and subcellular distribution of disease-associated prion protein in cerebellar organotypic slice cultures infected with scrapie.

Wolf H, Hossinger A, Fehlinger A, Büttner S, Sim V, McKenzie D, Vorberg IM.

Front Neurosci. 2015 Nov 4;9:410. doi: 10.3389/fnins.2015.00410. eCollection 2015.


Modulation of Glycosaminoglycans Affects PrPSc Metabolism but Does Not Block PrPSc Uptake.

Wolf H, Graßmann A, Bester R, Hossinger A, Möhl C, Paulsen L, Groschup MH, Schätzl H, Vorberg I.

J Virol. 2015 Oct;89(19):9853-64. doi: 10.1128/JVI.01276-15. Epub 2015 Jul 22.


Prion-induced and spontaneous formation of transmissible toxicity in PrP transgenic Drosophila.

Thackray AM, Di Y, Zhang C, Wolf H, Pradl L, Vorberg I, Andréoletti O, Bujdoso R.

Biochem J. 2014 Oct 1;463(1):31-40. doi: 10.1042/BJ20140129.


Prions Ex Vivo: What Cell Culture Models Tell Us about Infectious Proteins.

Krauss S, Vorberg I.

Int J Cell Biol. 2013;2013:704546. doi: 10.1155/2013/704546. Epub 2013 Oct 26. Review.


Life cycle of cytosolic prions.

Hofmann J, Vorberg I.

Prion. 2013 Sep-Oct;7(5):369-77. doi: 10.4161/pri.26414. Epub 2013 Sep 10. Review.


Cell-to-cell propagation of infectious cytosolic protein aggregates.

Hofmann JP, Denner P, Nussbaum-Krammer C, Kuhn PH, Suhre MH, Scheibel T, Lichtenthaler SF, Schätzl HM, Bano D, Vorberg IM.

Proc Natl Acad Sci U S A. 2013 Apr 9;110(15):5951-6. doi: 10.1073/pnas.1217321110. Epub 2013 Mar 18.


Cellular aspects of prion replication in vitro.

Grassmann A, Wolf H, Hofmann J, Graham J, Vorberg I.

Viruses. 2013 Jan 22;5(1):374-405. doi: 10.3390/v5010374. Review.


Creutzfeldt-Jakob disease and mad cows: lessons learnt from yeast cells.

Hofmann J, Wolf H, Grassmann A, Arndt V, Graham J, Vorberg I.

Swiss Med Wkly. 2012 Jan 24;142:w13505. doi: 10.4414/smw.2012.13505. eCollection 2012. Review.


Effect of hydrophobic mutations in the H2-H3 subdomain of prion protein on stability and conversion in vitro and in vivo.

Hafner-Bratkovič I, Gaedtke L, Ondracka A, Veranič P, Vorberg I, Jerala R.

PLoS One. 2011;6(9):e24238. doi: 10.1371/journal.pone.0024238. Epub 2011 Sep 1.


Proteasomal dysfunction and endoplasmic reticulum stress enhance trafficking of prion protein aggregates through the secretory pathway and increase accumulation of pathologic prion protein.

Nunziante M, Ackermann K, Dietrich K, Wolf H, Gädtke L, Gilch S, Vorberg I, Groschup M, Schätzl HM.

J Biol Chem. 2011 Sep 30;286(39):33942-53. doi: 10.1074/jbc.M111.272617. Epub 2011 Aug 11.


Globular domain of the prion protein needs to be unlocked by domain swapping to support prion protein conversion.

Hafner-Bratkovic I, Bester R, Pristovsek P, Gaedtke L, Veranic P, Gaspersic J, Mancek-Keber M, Avbelj M, Polymenidou M, Julius C, Aguzzi A, Vorberg I, Jerala R.

J Biol Chem. 2011 Apr 8;286(14):12149-56. doi: 10.1074/jbc.M110.213926. Epub 2011 Feb 15.


Prion protein interaction with stress-inducible protein 1 enhances neuronal protein synthesis via mTOR.

Roffé M, Beraldo FH, Bester R, Nunziante M, Bach C, Mancini G, Gilch S, Vorberg I, Castilho BA, Martins VR, Hajj GN.

Proc Natl Acad Sci U S A. 2010 Jul 20;107(29):13147-52. doi: 10.1073/pnas.1000784107. Epub 2010 Jul 6.


Tetracysteine-tagged prion protein allows discrimination between the native and converted forms.

Gaspersic J, Hafner-Bratkovic I, Stephan M, Veranic P, Bencina M, Vorberg I, Jerala R.

FEBS J. 2010 May;277(9):2038-50. doi: 10.1111/j.1742-4658.2010.07619.x. Epub 2010 Mar 19.


Prion-like propagation of cytosolic protein aggregates: insights from cell culture models.

Krammer C, Schätzl HM, Vorberg I.

Prion. 2009 Oct-Dec;3(4):206-12. Epub 2009 Oct 4. Review.


Inhibition of cholesterol recycling impairs cellular PrP(Sc) propagation.

Gilch S, Bach C, Lutzny G, Vorberg I, Schätzl HM.

Cell Mol Life Sci. 2009 Dec;66(24):3979-91. doi: 10.1007/s00018-009-0158-4. Epub 2009 Oct 13.


Prion-induced activation of cholesterogenic gene expression by Srebp2 in neuronal cells.

Bach C, Gilch S, Rost R, Greenwood AD, Horsch M, Hajj GN, Brodesser S, Facius A, Schädler S, Sandhoff K, Beckers J, Leib-Mösch C, Schätzl HM, Vorberg I.

J Biol Chem. 2009 Nov 6;284(45):31260-9. doi: 10.1074/jbc.M109.004382. Epub 2009 Sep 11.


Therapy in prion diseases: from molecular and cellular biology to therapeutic targets.

Krammer C, Vorberg I, Schätzl HM, Gilch S.

Infect Disord Drug Targets. 2009 Feb;9(1):3-14.


Dynamic interactions of Sup35p and PrP prion protein domains modulate aggregate nucleation and seeding.

Krammer C, Kremmer E, Schätzl HM, Vorberg I.

Prion. 2008 Jul-Sep;2(3):99-106.


The yeast Sup35NM domain propagates as a prion in mammalian cells.

Krammer C, Kryndushkin D, Suhre MH, Kremmer E, Hofmann A, Pfeifer A, Scheibel T, Wickner RB, Schätzl HM, Vorberg I.

Proc Natl Acad Sci U S A. 2009 Jan 13;106(2):462-7. doi: 10.1073/pnas.0811571106. Epub 2008 Dec 29.


Acute cellular uptake of abnormal prion protein is cell type and scrapie-strain independent.

Greil CS, Vorberg IM, Ward AE, Meade-White KD, Harris DA, Priola SA.

Virology. 2008 Sep 30;379(2):284-93. doi: 10.1016/j.virol.2008.07.006. Epub 2008 Aug 8.


Endocytosis of prion protein is required for ERK1/2 signaling induced by stress-inducible protein 1.

Caetano FA, Lopes MH, Hajj GN, Machado CF, Pinto Arantes C, Magalhães AC, Vieira Mde P, Américo TA, Massensini AR, Priola SA, Vorberg I, Gomez MV, Linden R, Prado VF, Martins VR, Prado MA.

J Neurosci. 2008 Jun 25;28(26):6691-702. doi: 10.1523/JNEUROSCI.1701-08.2008.


The novel sorting nexin SNX33 interferes with cellular PrP formation by modulation of PrP shedding.

Heiseke A, Schöbel S, Lichtenthaler SF, Vorberg I, Groschup MH, Kretzschmar H, Schätzl HM, Nunziante M.

Traffic. 2008 Jul;9(7):1116-29. doi: 10.1111/j.1600-0854.2008.00750.x. Epub 2008 Apr 18.


Prion protein/protein interactions: fusion with yeast Sup35p-NM modulates cytosolic PrP aggregation in mammalian cells.

Krammer C, Suhre MH, Kremmer E, Diemer C, Hess S, Schätzl HM, Scheibel T, Vorberg I.

FASEB J. 2008 Mar;22(3):762-73. Epub 2007 Oct 10.


Scrapie infection of prion protein-deficient cell line upon ectopic expression of mutant prion proteins.

Maas E, Geissen M, Groschup MH, Rost R, Onodera T, Schätzl H, Vorberg IM.

J Biol Chem. 2007 Jun 29;282(26):18702-10. Epub 2007 Apr 27.


Molecular aspects of disease pathogenesis in the transmissible spongiform encephalopathies.

Priola SA, Vorberg I.

Mol Biotechnol. 2006 May;33(1):71-88. Review.


Prion infection influences murine endogenous retrovirus expression in neuronal cells.

Stengel A, Bach C, Vorberg I, Frank O, Gilch S, Lutzny G, Seifarth W, Erfle V, Maas E, Schätzl H, Leib-Mösch C, Greenwood AD.

Biochem Biophys Res Commun. 2006 May 12;343(3):825-31. Epub 2006 Mar 15.


DNA aptamers that bind to PrP(C) and not PrP(Sc) show sequence and structure specificity.

Takemura K, Wang P, Vorberg I, Surewicz W, Priola SA, Kanthasamy A, Pottathil R, Chen SG, Sreevatsan S.

Exp Biol Med (Maywood). 2006 Feb;231(2):204-14. Erratum in: Exp Biol Med (Maywood). 2006 Apr;231(4):485.


Species barriers in prion diseases--brief review.

Moore RA, Vorberg I, Priola SA.

Arch Virol Suppl. 2005;(19):187-202. Review.


Cell line dependent RNA expression profiles of prion-infected mouse neuronal cells.

Greenwood AD, Horsch M, Stengel A, Vorberg I, Lutzny G, Maas E, Schädler S, Erfle V, Beckers J, Schätzl H, Leib-Mösch C.

J Mol Biol. 2005 Jun 10;349(3):487-500. Epub 2005 Apr 12.


Identification of possible animal origins of prion disease in human beings.

Priola SA, Vorberg I.

Lancet. 2004 Jun 19;363(9426):2013-4. No abstract available.


Molecular aspects of disease pathogenesis in the transmissible spongiform encephalopathies.

Priola SA, Vorberg I.

Methods Mol Biol. 2004;268:517-40. Review.


Acute formation of protease-resistant prion protein does not always lead to persistent scrapie infection in vitro.

Vorberg I, Raines A, Priola SA.

J Biol Chem. 2004 Jul 9;279(28):29218-25. Epub 2004 May 7.


Susceptibility of common fibroblast cell lines to transmissible spongiform encephalopathy agents.

Vorberg I, Raines A, Story B, Priola SA.

J Infect Dis. 2004 Feb 1;189(3):431-9. Epub 2004 Jan 21.


Multiple amino acid residues within the rabbit prion protein inhibit formation of its abnormal isoform.

Vorberg I, Groschup MH, Pfaff E, Priola SA.

J Virol. 2003 Feb;77(3):2003-9.


Molecular basis of scrapie strain glycoform variation.

Vorberg I, Priola SA.

J Biol Chem. 2002 Sep 27;277(39):36775-81. Epub 2002 Jul 23.

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