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Structural Transition, Function and Dysfunction of TDP-43 in Neurodegenerative Diseases.

Afroz T, Pérez-Berlanga M, Polymenidou M.

Chimia (Aarau). 2019 May 29;73(6):380-390. doi: 10.2533/chimia.2019.380. Review.


The Solution Structure of FUS Bound to RNA Reveals a Bipartite Mode of RNA Recognition with Both Sequence and Shape Specificity.

Loughlin FE, Lukavsky PJ, Kazeeva T, Reber S, Hock EM, Colombo M, Von Schroetter C, Pauli P, Cléry A, Mühlemann O, Polymenidou M, Ruepp MD, Allain FH.

Mol Cell. 2019 Feb 7;73(3):490-504.e6. doi: 10.1016/j.molcel.2018.11.012. Epub 2018 Dec 20.


TDP-43 extracted from frontotemporal lobar degeneration subject brains displays distinct aggregate assemblies and neurotoxic effects reflecting disease progression rates.

Laferrière F, Maniecka Z, Pérez-Berlanga M, Hruska-Plochan M, Gilhespy L, Hock EM, Wagner U, Afroz T, Boersema PJ, Barmettler G, Foti SC, Asi YT, Isaacs AM, Al-Amoudi A, Lewis A, Stahlberg H, Ravits J, De Giorgi F, Ichas F, Bezard E, Picotti P, Lashley T, Polymenidou M.

Nat Neurosci. 2019 Jan;22(1):65-77. doi: 10.1038/s41593-018-0294-y. Epub 2018 Dec 17.


Disease-modifying effects of metabolic perturbations in ALS/FTLD.

Jawaid A, Khan R, Polymenidou M, Schulz PE.

Mol Neurodegener. 2018 Dec 4;13(1):63. doi: 10.1186/s13024-018-0294-0. Review.


Biology/Disease-Driven Initiative on Protein-Aggregation Diseases of the Human Proteome Project: Goals and Progress to Date.

Boersema PJ, Melnik A, Hazenberg BPC, Rezeli M, Marko-Varga G, Kamiie J, Portelius E, Blennow K, Zubarev RA, Polymenidou M, Picotti P.

J Proteome Res. 2018 Dec 7;17(12):4072-4084. doi: 10.1021/acs.jproteome.8b00401. Epub 2018 Sep 28.


Memory Decline and Its Reversal in Aging and Neurodegeneration Involve miR-183/96/182 Biogenesis.

Jawaid A, Woldemichael BT, Kremer EA, Laferriere F, Gaur N, Afroz T, Polymenidou M, Mansuy IM.

Mol Neurobiol. 2019 May;56(5):3451-3462. doi: 10.1007/s12035-018-1314-3. Epub 2018 Aug 20.


Hypertonic Stress Causes Cytoplasmic Translocation of Neuronal, but Not Astrocytic, FUS due to Impaired Transportin Function.

Hock EM, Maniecka Z, Hruska-Plochan M, Reber S, Laferrière F, Sahadevan M K S, Ederle H, Gittings L, Pelkmans L, Dupuis L, Lashley T, Ruepp MD, Dormann D, Polymenidou M.

Cell Rep. 2018 Jul 24;24(4):987-1000.e7. doi: 10.1016/j.celrep.2018.06.094.


The RNA face of phase separation.

Polymenidou M.

Science. 2018 May 25;360(6391):859-860. doi: 10.1126/science.aat8028. No abstract available.


Protein Phase Separation: A New Phase in Cell Biology.

Boeynaems S, Alberti S, Fawzi NL, Mittag T, Polymenidou M, Rousseau F, Schymkowitz J, Shorter J, Wolozin B, Van Den Bosch L, Tompa P, Fuxreiter M.

Trends Cell Biol. 2018 Jun;28(6):420-435. doi: 10.1016/j.tcb.2018.02.004. Epub 2018 Mar 27. Review.


Split GFP technologies to structurally characterize and quantify functional biomolecular interactions of FTD-related proteins.

Foglieni C, Papin S, Salvadè A, Afroz T, Pinton S, Pedrioli G, Ulrich G, Polymenidou M, Paganetti P.

Sci Rep. 2017 Oct 25;7(1):14013. doi: 10.1038/s41598-017-14459-w.


Functional and dynamic polymerization of the ALS-linked protein TDP-43 antagonizes its pathologic aggregation.

Afroz T, Hock EM, Ernst P, Foglieni C, Jambeau M, Gilhespy LAB, Laferriere F, Maniecka Z, Plückthun A, Mittl P, Paganetti P, Allain FHT, Polymenidou M.

Nat Commun. 2017 Jun 29;8(1):45. doi: 10.1038/s41467-017-00062-0.


Protease resistance of infectious prions is suppressed by removal of a single atom in the cellular prion protein.

Leske H, Hornemann S, Herrmann US, Zhu C, Dametto P, Li B, Laferriere F, Polymenidou M, Pelczar P, Reimann RR, Schwarz P, Rushing EJ, Wüthrich K, Aguzzi A.

PLoS One. 2017 Feb 16;12(2):e0170503. doi: 10.1371/journal.pone.0170503. eCollection 2017.


Biological Spectrum of Amyotrophic Lateral Sclerosis Prions.

Polymenidou M, Cleveland DW.

Cold Spring Harb Perspect Med. 2017 Nov 1;7(11). pii: a024133. doi: 10.1101/cshperspect.a024133. Review.


Prion-like propagation as a pathogenic principle in frontotemporal dementia.

Hock EM, Polymenidou M.

J Neurochem. 2016 Aug;138 Suppl 1:163-83. doi: 10.1111/jnc.13668. Review.


New and emerging roles of small RNAs in neurodegeneration, muscle, cardiovascular and inflammatory diseases.

Hruska-Plochan M, Li B, Kyburz D, Krützfeld J, Landmesser U, Aguzzi A, Polymenidou M.

Swiss Med Wkly. 2015 Sep 16;145:w14192. doi: 10.4414/smw.2015.14192. eCollection 2015. Review.


Wild type human TDP-43 potentiates ALS-linked mutant TDP-43 driven progressive motor and cortical neuron degeneration with pathological features of ALS.

Mitchell JC, Constable R, So E, Vance C, Scotter E, Glover L, Hortobagyi T, Arnold ES, Ling SC, McAlonis M, Da Cruz S, Polymenidou M, Tessarolo L, Cleveland DW, Shaw CE.

Acta Neuropathol Commun. 2015 Jun 25;3:36. doi: 10.1186/s40478-015-0212-4.


From nucleation to widespread propagation: A prion-like concept for ALS.

Maniecka Z, Polymenidou M.

Virus Res. 2015 Sep 2;207:94-105. doi: 10.1016/j.virusres.2014.12.032. Epub 2015 Feb 2. Review.


Advances and challenges in understanding the multifaceted pathogenesis of amyotrophic lateral sclerosis.

Laferriere F, Polymenidou M.

Swiss Med Wkly. 2015 Jan 30;145:w14054. doi: 10.4414/smw.2015.14054. eCollection 2015. Review.


Targeted degradation of sense and antisense C9orf72 RNA foci as therapy for ALS and frontotemporal degeneration.

Lagier-Tourenne C, Baughn M, Rigo F, Sun S, Liu P, Li HR, Jiang J, Watt AT, Chun S, Katz M, Qiu J, Sun Y, Ling SC, Zhu Q, Polymenidou M, Drenner K, Artates JW, McAlonis-Downes M, Markmiller S, Hutt KR, Pizzo DP, Cady J, Harms MB, Baloh RH, Vandenberg SR, Yeo GW, Fu XD, Bennett CF, Cleveland DW, Ravits J.

Proc Natl Acad Sci U S A. 2013 Nov 19;110(47):E4530-9. doi: 10.1073/pnas.1318835110. Epub 2013 Oct 29.


Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis.

Ling SC, Polymenidou M, Cleveland DW.

Neuron. 2013 Aug 7;79(3):416-38. doi: 10.1016/j.neuron.2013.07.033. Review.


ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43.

Arnold ES, Ling SC, Huelga SC, Lagier-Tourenne C, Polymenidou M, Ditsworth D, Kordasiewicz HB, McAlonis-Downes M, Platoshyn O, Parone PA, Da Cruz S, Clutario KM, Swing D, Tessarollo L, Marsala M, Shaw CE, Yeo GW, Cleveland DW.

Proc Natl Acad Sci U S A. 2013 Feb 19;110(8):E736-45. doi: 10.1073/pnas.1222809110. Epub 2013 Feb 4.


Structural studies on the folded domain of the human prion protein bound to the Fab fragment of the antibody POM1.

Baral PK, Wieland B, Swayampakula M, Polymenidou M, Rahman MH, Kav NN, Aguzzi A, James MN.

Acta Crystallogr D Biol Crystallogr. 2012 Nov;68(Pt 11):1501-12. doi: 10.1107/S0907444912037328. Epub 2012 Oct 18.


Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAs.

Lagier-Tourenne C, Polymenidou M, Hutt KR, Vu AQ, Baughn M, Huelga SC, Clutario KM, Ling SC, Liang TY, Mazur C, Wancewicz E, Kim AS, Watt A, Freier S, Hicks GG, Donohue JP, Shiue L, Bennett CF, Ravits J, Cleveland DW, Yeo GW.

Nat Neurosci. 2012 Nov;15(11):1488-97. doi: 10.1038/nn.3230. Epub 2012 Sep 30.


Prion-like spread of protein aggregates in neurodegeneration.

Polymenidou M, Cleveland DW.

J Exp Med. 2012 May 7;209(5):889-93. doi: 10.1084/jem.20120741. Review.


Misregulated RNA processing in amyotrophic lateral sclerosis.

Polymenidou M, Lagier-Tourenne C, Hutt KR, Bennett CF, Cleveland DW, Yeo GW.

Brain Res. 2012 Jun 26;1462:3-15. doi: 10.1016/j.brainres.2012.02.059. Epub 2012 Mar 3. Review.


Crystallization and preliminary X-ray diffraction analysis of prion protein bound to the Fab fragment of the POM1 antibody.

Baral PK, Wieland B, Swayampakula M, Polymenidou M, Aguzzi A, Kav NN, James MN.

Acta Crystallogr Sect F Struct Biol Cryst Commun. 2011 Oct 1;67(Pt 10):1211-3. doi: 10.1107/S1744309111026273. Epub 2011 Sep 24.


The seeds of neurodegeneration: prion-like spreading in ALS.

Polymenidou M, Cleveland DW.

Cell. 2011 Oct 28;147(3):498-508. doi: 10.1016/j.cell.2011.10.011.


Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43.

Polymenidou M, Lagier-Tourenne C, Hutt KR, Huelga SC, Moran J, Liang TY, Ling SC, Sun E, Wancewicz E, Mazur C, Kordasiewicz H, Sedaghat Y, Donohue JP, Shiue L, Bennett CF, Yeo GW, Cleveland DW.

Nat Neurosci. 2011 Apr;14(4):459-68. doi: 10.1038/nn.2779. Epub 2011 Feb 27.


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.


Atypical prion protein conformation in familial prion disease with PRNP P105T mutation.

Polymenidou M, Prokop S, Jung HH, Hewer E, Peretz D, Moos R, Tolnay M, Aguzzi A.

Brain Pathol. 2011 Mar;21(2):209-14. doi: 10.1111/j.1750-3639.2010.00439.x. Epub 2010 Sep 28.


TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration.

Lagier-Tourenne C, Polymenidou M, Cleveland DW.

Hum Mol Genet. 2010 Apr 15;19(R1):R46-64. doi: 10.1093/hmg/ddq137. Epub 2010 Apr 15. Review.


Non-cell autonomous toxicity in neurodegenerative disorders: ALS and beyond.

Ilieva H, Polymenidou M, Cleveland DW.

J Cell Biol. 2009 Dec 14;187(6):761-72. doi: 10.1083/jcb.200908164. Review.


Species and strain glycosylation patterns of PrPSc.

Xanthopoulos K, Polymenidou M, Bellworthy SJ, Benestad SL, Sklaviadis T.

PLoS One. 2009 May 20;4(5):e5633. doi: 10.1371/journal.pone.0005633.


Lymphotoxin-dependent prion replication in inflammatory stromal cells of granulomas.

Heikenwalder M, Kurrer MO, Margalith I, Kranich J, Zeller N, Haybaeck J, Polymenidou M, Matter M, Bremer J, Jackson WS, Lindquist S, Sigurdson CJ, Aguzzi A.

Immunity. 2008 Dec 19;29(6):998-1008. doi: 10.1016/j.immuni.2008.10.014.


Olfactory behavior and physiology are disrupted in prion protein knockout mice.

Le Pichon CE, Valley MT, Polymenidou M, Chesler AT, Sagdullaev BT, Aguzzi A, Firestein S.

Nat Neurosci. 2009 Jan;12(1):60-9. doi: 10.1038/nn.2238. Epub 2008 Dec 21.


The POM monoclonals: a comprehensive set of antibodies to non-overlapping prion protein epitopes.

Polymenidou M, Moos R, Scott M, Sigurdson C, Shi YZ, Yajima B, Hafner-Bratkovic I, Jerala R, Hornemann S, Wuthrich K, Bellon A, Vey M, Garen G, James MN, Kav N, Aguzzi A.

PLoS One. 2008;3(12):e3872. doi: 10.1371/journal.pone.0003872. Epub 2008 Dec 8.


Motor neuron disease: The curious ways of ALS.

Polymenidou M, Cleveland DW.

Nature. 2008 Jul 17;454(7202):284-5. doi: 10.1038/454284a. No abstract available.


Follicular dendritic cells control engulfment of apoptotic bodies by secreting Mfge8.

Kranich J, Krautler NJ, Heinen E, Polymenidou M, Bridel C, Schildknecht A, Huber C, Kosco-Vilbois MH, Zinkernagel R, Miele G, Aguzzi A.

J Exp Med. 2008 Jun 9;205(6):1293-302. doi: 10.1084/jem.20071019. Epub 2008 May 19.


Canine MDCK cell lines are refractory to infection with human and mouse prions.

Polymenidou M, Trusheim H, Stallmach L, Moos R, Julius C, Miele G, Lenz-Bauer C, Aguzzi A.

Vaccine. 2008 May 19;26(21):2601-14. doi: 10.1016/j.vaccine.2008.03.035. Epub 2008 Apr 9.


Prion strain discrimination using luminescent conjugated polymers.

Sigurdson CJ, Nilsson KP, Hornemann S, Manco G, Polymenidou M, Schwarz P, Leclerc M, Hammarström P, Wüthrich K, Aguzzi A.

Nat Methods. 2007 Dec;4(12):1023-30. Epub 2007 Nov 18.


Insights into prion strains and neurotoxicity.

Aguzzi A, Heikenwalder M, Polymenidou M.

Nat Rev Mol Cell Biol. 2007 Jul;8(7):552-61. Review.


Strain fidelity of chronic wasting disease upon murine adaptation.

Sigurdson CJ, Manco G, Schwarz P, Liberski P, Hoover EA, Hornemann S, Polymenidou M, Miller MW, Glatzel M, Aguzzi A.

J Virol. 2006 Dec;80(24):12303-11. Epub 2006 Oct 4.


Reconstructing prions: fibril assembly from simple yeast to complex mammals.

Sigurdson C, Polymenidou M, Aguzzi A.

Neurodegener Dis. 2005;2(1):1-5. Review.


Coexistence of multiple PrPSc types in individuals with Creutzfeldt-Jakob disease.

Polymenidou M, Stoeck K, Glatzel M, Vey M, Bellon A, Aguzzi A.

Lancet Neurol. 2005 Dec;4(12):805-14. Erratum in: Lancet Neurol. 2005 Dec;4(12):795.


Paracrine inhibition of prion propagation by anti-PrP single-chain Fv miniantibodies.

Donofrio G, Heppner FL, Polymenidou M, Musahl C, Aguzzi A.

J Virol. 2005 Jul;79(13):8330-8.


Humoral immune response to native eukaryotic prion protein correlates with anti-prion protection.

Polymenidou M, Heppner FL, Pellicioli EC, Urich E, Miele G, Braun N, Wopfner F, Schätzl HM, Becher B, Aguzzi A.

Proc Natl Acad Sci U S A. 2004 Oct 5;101 Suppl 2:14670-6. Epub 2004 Aug 3.


Lymphoid follicle destruction and immunosuppression after repeated CpG oligodeoxynucleotide administration.

Heikenwalder M, Polymenidou M, Junt T, Sigurdson C, Wagner H, Akira S, Zinkernagel R, Aguzzi A.

Nat Med. 2004 Feb;10(2):187-92. Epub 2004 Jan 25.


Mammalian prion biology: one century of evolving concepts.

Aguzzi A, Polymenidou M.

Cell. 2004 Jan 23;116(2):313-27. Review.


A short purification process for quantitative isolation of PrPSc from naturally occurring and experimental transmissible spongiform encephalopathies.

Polymenidou M, Verghese-Nikolakaki S, Groschup M, Chaplin MJ, Stack MJ, Plaitakis A, Sklaviadis T.

BMC Infect Dis. 2002 Oct 8;2:23.


Expression of the prion protein in the rat forebrain--an immunohistochemical study.

Verghese-Nikolakaki S, Michaloudi H, Polymenidou M, Groschup MH, Papadopoulos GC, Sklaviadis T.

Neurosci Lett. 1999 Sep 3;272(1):9-12.


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