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Recent Prion Publications

Vázquez-Fernández E, Vos MR, Afanasyev P, Cebey L, Sevillano AM, Vidal E, Rosa I, Renault L, Ramos A, Peters PJ, Fernández JJ, van Heel M, Young HS, Requena JR, Wille H. "The Structural Architecture of an Infectious Mammalian Prion Using Electron Cryomicroscopy." PLoS Pathog. 2016 Sep 8;12(9):e1005835. doi: 10.1371/journal.ppat.1005835. eCollection 2016 Sep. PMID: 27606840

Beraldo FH, Ostapchenko VG, Caetano FA, Guimaraes AL, Ferretti GD, Daude N, Bertram L, Nogueira KO, Silva JL, Westaway D, Cashman NR, Martins VR, Prado VF, Prado MA. "Regulation of Amyloid β oligomer binding to neurons and neurotoxicity by the complex prion protein/mGluR5." J Biol Chem. 2016 Aug 25. pii: jbc.M116.738286. PMID: 27563063

Herbst A, Wanagat J, Cheema N, Widjaja K, McKenzie D, Aiken JM. "Latent mitochondrial DNA deletion mutations drive muscle fiber loss at old age." Aging Cell. 2016 Aug 25. doi: 10.1111/acel.12520. PMID: 27561813

Breid S, Bernis ME, Babila JT, Garca MC, Wille H, Tamgüney G. "Neuroinvasion of α-Synuclein Prionoids after Intraperitoneal and Intraglossal Inoculation." J Virol. 2016 Aug 3. pii: JVI.01399-16. PMID: 27489279

Leighton PL, Allison WT. "Protein Misfolding in Prion and Prion-Like Diseases: Reconsidering a Required Role for Protein Loss-of-Function." J Alzheimers Dis. 2016 Jul 6;54(1):3-29. doi: 10.3233/JAD-160361. PMID: 27392869

Gupta AN, Neupane K, Rezajooei N, Cortez LM, Sim VL, Woodside MT. "Pharmacological chaperone reshapes the energy landscape for folding and aggregation of the prion protein." Nat Commun. 2016 Jun 27;7:12058. doi: 10.1038/ncomms12058. PMID: 27346148

Fang C, Imberdis T, Garza MC, Wille H, Harris DA. "A Neuronal Culture System to Detect Prion Synaptotoxicity." PLoS Pathog. 2016 May 26;12(5):e1005623. doi: 10.1371/journal.ppat.1005623. eCollection 2016 May. PMID: 27227882

Puig B, Altmeppen HC, Ulbrich S, Linsenmeier L, Krasemann S, Chakroun K, Acevedo-Morantes CY, Wille H, Tatzelt J, Glatzel M. "Secretory pathway retention of mutant prion protein induces p38-MAPK activation and lethal disease in mice." Sci Rep. 2016 Apr 27;6:24970. doi: 10.1038/srep24970. PMID: 27117504

Wille H. "The Detection of Infectious Prions: In Vitro Conversion Assays Change the (Folding) Landscape." J Mol Diagn. 2016 May;18(3):329-30. doi: 10.1016/j.jmoldx.2016.03.001. Epub 2016 Mar 25. PMID: 27020948

Kang SG, Kim C, Cortez LM, Carmen Garza M, Yang J, Wille H, Sim VL, Westaway D, McKenzie D, Aiken J. "Toll-like receptor-mediated immune response inhibits prion propagation." Glia. 2016 Jun;64(6):937-51. doi: 10.1002/glia.22973. Epub 2016 Feb 16. PMID: 26880394

Kimura R, MacTavish D, Yang J, Westaway D, Jhamandas JH. "Pramlintide Antagonizes Beta Amyloid (Aβ)- and Human Amylin-Induced Depression of Hippocampal Long-Term Potentiation." Mol Neurobiol. 2016 Jan 15. [Epub ahead of print] PMID: 26768593

Stocki P, Sawicki M, Mays CE, Hong SJ, Chapman DC, Westaway D, Williams DB. "Inhibition of the FKBP family of peptidyl prolyl isomerases induces abortive translocation and degradation of the cellular prion protein." Mol Biol Cell. 2016 Mar 1;27(5):757-67. doi: 10.1091/mbc.E15-10-0729. Epub 2016 Jan 13. PMID: 26764098

Wolf H, Hossinger A, Fehlinger A, Büttner S, Sim V, McKenzie D, Vorberg IM. "Deposition pattern and subcellular distribution of disease-associated prion protein in cerebellar organotypic slice cultures infected with scrapie." Front Neurosci. 2015 Nov 4;9:410. doi: 10.3389/fnins.2015.00410. eCollection 2015. PMID: 26581229

Daude N, Gapeshina H, Dong B, Winship I, Westaway D. "Neuroprotective properties of the PrP-like Shadoo glycoprotein assessed in the middle cerebral artery occlusion model of ischemia." Prion. 2015 Oct 30:0. PMID: 26516793

Mays CE, van der Merwe J, Kim C, Haldiman T, McKenzie D, Safar JG, Westaway D. "Prion infectivity plateaus and conversion to symptomatic disease originate from falling precursor levels and increased oligomeric PrPSc species." J Virol. 2015 Sep 30. pii: JVI.02142-15. PMID: 26423957

Duque Velásquez C, Kim C, Herbst A, Daude N, Garza MC, Wille H, Aiken J, McKenzie D. "Deer Prion Proteins Modulate the Emergence and Adaptation of Chronic Wasting Disease Strains." J Virol. 2015 Sep 30. pii: JVI.02010-15. PMID: 26423950

Cheema N, Herbst A, McKenzie D, Aiken JM. "Apoptosis and necrosis mediate skeletal muscle fiber loss in age-induced mitochondrial enzymatic abnormalities." Aging Cell. 2015 Sep 14. doi: 10.1111/acel.12399. PMID: 26365892

Westaway D. "Basic Prion Science "Spreads" Insight." PLoS Pathog. 2015 Jul 30;11(7):e1005092. doi: 10.1371/journal.ppat.1005092. eCollection 2015. PMID: 26226044

Herbst A, Ness A, Johnson CJ, McKenzie D, Aiken JM. "Transcriptomic responses to prion disease in rats." BMC Genomics. 2015 Sep 5;16(1):682. doi: 10.1186/s12864-015-1884-7. PMID: 26341492

Cortez LM, Campeau J, Norman G, Kalayil M, Van der Merwe J, McKenzie D, Sim VL. "Bile Acids Reduce Prion Conversion, Reduce Neuronal Loss, and Prolong Male Survival in Models of Prion Disease." J Virol. 2015 Aug;89(15):7660-72. PMID: 25972546

Ciric D, Richard CA, Moudjou M, Chapuis J, Sibille P, Daude N, Westaway D, Adrover Estelrich M, Béringue V, Martin D, Rezaei H. "Interaction between Shadoo and PrP affects the PrP folding pathway." J Virol. 2015 Apr 8. pii: JVI.03429-14. [Epub ahead of print] PMID: 25855735

Wan W, Wille H, Stöhr J, Kendall A, Bian W, McDonald M, Tiggelaar S, Watts JC, Prusiner SB, Stubbs G. "Structural Studies of Truncated Forms of the Prion Protein PrP." Biophys J. 2015 Mar 24;108(6):1548-54. doi: 10.1016/j.bpj.2015.01.008. PMID: 25809267

Levine DJ, Stöhr J, Falese LE, Ollesch J, Wille H, Prusiner SB, Long JR. "Mechanism of Scrapie Prion Precipitation with Phosphotungstate Anions." ACS Chem Biol. 2015 Feb 19. [Epub ahead of print] PMID: 25695325

Godsave SF, Peters PJ, Wille H. "Subcellular distribution of the prion protein in sickness and in health." Virus Res. 2015 Feb 12. pii: S0168-1702(15)00057-X. doi: 10.1016/j.virusres.2015.02.004. [Epub ahead of print] PMID: 25683509

Lau A, McDonald A, Daude N, Mays CE, Walter ED, Aglietti R, Mercer RC, Wohlgemuth S, van der Merwe J, Yang J, Gapeshina H, Kim C, Grams J, Shi B, Wille H, Balachandran A, Schmitt-Ulms G, Safar JG, Millhauser GL, Westaway D. "Octarepeat region flexibility impacts prion function, endoproteolysis and disease manifestation." EMBO Mol Med. 2015 Feb 6. pii: e201404588. doi: 10.15252/emmm.201404588. PMID: 25661904

van der Merwe J, Aiken J, Westaway D, McKenzie D. "The Standard Scrapie Cell Assay: Development, Utility and Prospects." Viruses. 2015 Jan 16;7(1):180-198. PMID: 25602372

Huc-Brandt S, Hieu N, Imberdis T, Cubedo N, Silhol M, Leighton PL, Domaschke T, Allison WT, Perrier V, Rossel M. "Zebrafish prion protein PrP2 controls collective migration process during lateral line sensory system development." PLoS One. 2014 Dec 1;9(12):e113331. doi: 10.1371/journal.pone.0113331. eCollection 2014. PMID: 25436888

Acevedo-Morantes CY, Wille H. "The structure of human prions: from biology to structural models-considerations and pitfalls." Viruses. 2014 Oct 20;6(10):3875-92. doi: 10.3390/v6103875. PMID: 25333467

Requena JR, Wille H. "The structure of the infectious prion protein: experimental data and molecular models." Prion. 2014 Jan-Feb;8(1):60-6. PMID: 24583975

Herbst A, Aiken JM, McKenzie D. "Replication of Prions in Differentiated Muscle Cells." Prion. 2014 Feb 11;8(2). PMID: 24518642

Mays CE, Kim C, Haldiman T, van der Merwe J, Lau A, Yang J, Grams J, Di Bari MA, Nonno R, Telling GC, Kong Q, Langeveld J, McKenzie D, Westaway D, Safar JG. "Prion disease tempo determined by host-dependent substrate reduction." J Clin Invest. 2014 Feb 3;124(2):847-58. doi: 10.1172/JCI72241. Epub 2014 Jan 16. PMID: 24430187

Mays CE, Coomaraswamy J, Watts JC, Yang J, Ko KW, Strome B, Mercer RC, Wohlgemuth SL, Schmitt-Ulms G, Westaway D. "Endoproteolytic Processing of the Mammalian Prion Glycoprotein Family." FEBS J. 2013 Nov 29. doi: 10.1111/febs.12654. PMID: 24286250

Herbst A, Banser P, Velasquez CD, Mays CE, Sim VL, Westaway D, Aiken JM, McKenzie D. "Infectious Prions Accumulate to High Levels in Non Proliferative C2C12 Myotubes." PLoS Pathog. 2013 Nov;9(11):e1003755. doi: 10.1371/journal.ppat.1003755. PMID: 24244171

Mercer RC, Ma L, Watts JC, Strome R, Wohlgemuth S, Yang J, Cashman NR, Coulthart MB, Schmitt-Ulms G, Jhamandas JH, Westaway D. "The prion protein modulates A-type K+ currents mediated by Kv4.2 complexes through Dipeptidyl Aminopeptidase-like protein 6." J Biol Chem. 2013 Nov 13. PMID: 24225951

Pocanschi CL, Ehsani S, Mehrabian M, Wille H, Reginold W, Trimble WS, Wang H, Yee A, Arrowsmith CH, Bozóky Z, Kay LE, Forman-Kay JD, Rini JM, Schmitt-Ulms G. "The ZIP5 Ectodomain Co-Localizes with PrP and May Acquire a PrP-Like Fold That Assembles into a Dimer." PLoS One. 2013 Sep 6;8(9):e72446. doi: 10.1371/journal.pone.0072446. PMID: 24039764

Pillay LM, Selland LG, Fleisch VC, Leighton PL, Cheng CS, Famulski JK, Ritzel RG, March LD, Wang H, Allison WT, Waskiewicz AJ. "Evaluating the Mutagenic Activity of Targeted Endonucleases Containing a Sharkey FokI Cleavage Domain Variant in Zebrafish." Zebrafish. 2013 Sep;10(3):353-64. doi: 10.1089/zeb.2012.0832. Epub 2013 Jun 19. PMID: 23781947

Fleisch VC, PLA Leighton, H Wang, LM Pillay, G Ritzel, G Bhinder, B Roy, DW Ali, KB Tierney, AJ Waskiewicz, WT Allison. "Targeted mutation of the gene encoding prion protein in zebrafish reveals a conserved role in modulating neuron excitability." Neurobiology of Disease. 2013 Jul;55:11-25. doi: 10.1016/j.nbd.2013.03.007. Epub 2013 Mar 21. PMID: 23523635

Godsave SF, Wille H, Pierson J, Prusiner SB, Peters PJ."Plasma membrane invaginations containing clusters of full-length PrP(Sc) are an early form of prion-associated neuropathology in vivo." Neurobiol Aging. 2013 Jun;34(6):1621-31. doi: 10.1016/j.neurobiolaging.2012.12.015. PMID: 23481568

Johnson CJ, Gilbert PU, Abrecht M, Baldwin KL, Russell RE, Pedersen JA, Aiken JM, McKenzie D."Low copper and high manganese levels in prion protein plaques." Viruses. 2013 Feb 11;5(2):654-62. doi: 10.3390/v5020654. PMID: 23435237

Cortez LM, Kumar J, Renault L, Young HS, Sim VL."Mouse prion protein polymorphism 108F/189V affects the kinetics of fibril formation and the response to seeding; evidence for a two step nucleation polymerization mechanism." J Biol Chem. 2013 Jan 2. PMID: 23283973

Darcy M. Kaiser, Moulinath Acharya1, Patricia L. A. Leighton, Hao Wang, Nathalie Daude, Serene Wohlgemuth, Beipei Shi, W. Ted Allison"Amyloid Beta Precursor Protein and Prion Protein Have a Conserved Interaction Affecting Cell Adhesion and CNS Development." PLoS One. 2012;7(12):e51305. doi: 10.1371/journal.pone.0051305. PMID: 23236467

Lau A, Mays CE, Genovesi S, Westaway D. "RGG Repeats of PrP-like Shadoo Protein Bind Nucleic Acids." Biochemistry. 2012 Nov 2. PMID: 23121093

Daude N, Westaway D. "Shadoo/PrP (Sprn ( 0/0) /Prnp ( 0/0) ) double knockout mice: More than zeroes." Prion. 2012 Aug 28;7(1). PMID: 22929230

Westaway D, Jhamandas JH. "The P's and Q's of cellular PrP-Aβ interactions." Prion. 2012 Sep 1;6(4):359-63. Epub 2012 Aug 9. PMID: 22874673

Mays CE, Joy S, Li L, Yu L, Genovesi S, West FG, Westaway D. "Prion inhibition with multivalent PrPSc binding compounds." Biomaterials. 2012 Oct;33(28):6808-22. Epub 2012 Jun 28. PMID: 22748770

Ehsani S, Salehzadeh A, Huo H, Reginold W, Pocanschi CL, Ren H, Wang H, So K, Sato C, Mehrabian M, Strome R, Trimble WS, Hazrati LN, Rogaeva E, Westaway D, Carlson GA, Schmitt-Ulms G. "LIV-1 ZIP ectodomain shedding in prion-infected mice resembles cellular response to transition metal starvation." J Mol Biol. 2012 Sep 28;422(4):556-74. doi: 10.1016/j.jmb.2012.06.003. Epub 2012 Jun 9. PMID: 22687393

Wan W, Wille H, Stöhr J, Baxa U, Prusiner SB, Stubbs G. "Degradation of fungal prion HET-s(218-289) induces formation of a generic amyloid fold." Biophys J. 2012 May 16;102(10):2339-44. doi: 10.1016/j.bpj.2012.04.011. Epub 2012 May 15. PMID: 22677387

Daude N, Wohlgemuth S, Brown R, Pitstick R, Gapeshina H, Yang J, Carlson GA, Westaway D. "Knockout of the prion protein (PrP)-like Sprn gene does not produce embryonic lethality in combination with PrPC-deficiency." PNAS. 2012 May 22. PMID: 22619325

Johnson CJ, Aiken JM, McKenzie D, Samuel MD, Pedersen JA. "Highly Efficient Amplification of Chronic Wasting Disease Agent by Protein Misfolding Cyclic Amplification with Beads (PMCAb)." PLoS One. 2012;7(4):e35383. Epub 2012 Apr 13. PMID: 22514738

Sim VL. "Prion disease: chemotherapeutic strategies." Infect Disord Drug Targets. 2012 Apr;12(2):144-60. PMID: 22420513

Westaway D, Genovesi S, Daude N, Brown R, Lau A, Lee I, Mays CE, Coomaraswamy J, Canine B, Pitstick R, Herbst A, Yang J, Ko KW, Schmitt-Ulms G, Dearmond SJ, McKenzie D, Hood L, Carlson GA. "Down-Regulation of Shadoo in Prion Infections Traces a Pre-Clinical Event Inversely Related to PrP Accumulation." PLoS Pathog. 2011 Nov;7(11):e1002391. Epub 2011 Nov 17. PMID: 22114562

Westaway D, Daude N, Wohlgemuth S, Harrison P. "The PrP-like proteins Shadoo and Doppel." Top Curr Chem. 2011;305:225-56. PMID: 21728138

Kang SG, Roh YM, Lau A, Westaway D, McKenzie D, Aiken J, Kim YS, Yoo HS. "Establishment and characterization of Prnp knockdown neuroblastoma cells using dual microRNA-mediated RNA interference." Prion. 2011 Apr-Jun;5(2):93-102. Epub 2011 Apr 1. PMID: 21494092

Wei X, Herbst A, Ma D, Aiken J, Li L."A quantitative proteomic approach to prion disease biomarker research: delving into the glycoproteome." J Proteome Res. 2011 Jun 3;10(6):2687-702. Epub 2011 Apr 25. PMID: 21469646

Johnson CJ, McKenzie D, Pedersen JA, Aiken JM."Meat and bone meal and mineral feed additives may increase the risk of oral prion disease transmission." J Toxicol Environ Health A. 2011;74(2-4):161-6. PMID: 21218345

Moody LR, Herbst AJ, Aiken JM."Upregulation of interferon-gamma-induced genes during prion infection." J Toxicol Environ Health A. 2011;74(2-4):146-53. PMID: 21218343

Wei, X., Herbst, A., Ma, D., Aiken, J., Li, L. "A Quantitative Proteomic Approach to Prion Disease Biomarker Research: Delving into the Glycoproteome." Journal of Proteome Research, April 6, 2011. PMID: 21469646

Chad J. Johnson, Allen Herbst, Camilo Duque-Velasquez, Joshua P. Vanderloo, Phil Bochsler, Rick Chappell, Debbie McKenzie "Prion Protein Polymorphisms Affect Chronic Wasting Disease Progression " PLoS ONE 6(3): e17450. doi:10.1371/journal.pone.0017450 Mar 2011. PMID: 21445256

Wong M, Toth J, Haney S, Krewski D, Leighton FA, Ricketts M, Westaway D, Cashman N. "PrioNet Canada: a network of centres of excellence for research on prion diseases--ongoing and future research directions." J Toxicol Environ Health A. 2011;74(2-4):73-87. PMID: 21218336

Moody, LR., Herbst, A., Aiken, JM. "Up-regulation of interferon-gamma induced genes during prion infection." Journal of Toxicology and Environmental Health, Part A 74(2) January 2011. PMID: 21218343

Daude N, Westaway D. "Biological properties of the PrP-like Shadoo protein." (pdf link) Front Biosci. 2011 Jan 1;16:1505-16. PMID: 21196244

Johnson, C.J., D. McKenzie, J.A. Pedersen and J.M. Aiken."Meat and bone meal and mineral feed additives may increase risk of oral prion disease transmission." 2010 J. Toxicol Environ Health, Part A 74(2) January 2011. PMID: 21218345

Allison WT "Preface: zebrafish models of neurology." Biochim Biophys Acta. 2011 Mar;1812(3):333-4. doi: 10.1016/j.bbadis.2010.11.004. Epub 2010 Nov 27. PMID: 21115091

Fleisch VC, Fraser B, Allison WT "Investigating regeneration and functional integration of CNS neurons: lessons from zebrafish genetics and other fish species." Biochim Biophys Acta. 2011 Mar;1812(3):364-80. doi: 10.1016/j.bbadis.2010.10.012. Epub 2010 Oct 28. PMID: 21044883

Kwai Alier, Zongming Li, David MacTavish, David Westaway and Jack H. Jhamandas "Ionic mechanisms of action of prion protein fragment, PrP(106-126), in rat basal forebrain neurons" J Neurosci Res. 2010 Aug 1;88(10):2217-27. PMID: 20175205

Jacobson KH, Lee S, Somerville RA, McKenzie D, Benson CH, Pedersen JA."Transport of the pathogenic prion protein through soils." J Environ Qual. 2010 Jul-Aug;39(4):1145-52. PMID: 20830901

Angers, R.C., H-E. Kang, D. Napier, S. Browning, T. Seward, C. Mathiason, A. Balachandran, D. McKenzie, J. Castilla, C. Soto, J. Jewell, C.G. Graham, E.A. Hoover and G.C. Telling."Prion strain mutation determined by prion protein conformational compatibility and primary structure." Science. 2010 May 28;328(5982):1111-2. PMID: 20466881

Nathalie Daude, Vivian Ng, Joel C. Watts, Sacha Genovesi, John Paul Glaves, Serene Wohlgemuth, Gerold Schmitt-Ulms, Howard Young, JoAnne McLaurin, Paul E. Fraser and David Westaway "Wild-type Shadoo proteins convert to amyloid-like forms under native conditions" J. Neurochem. 2010 Apr;113(1):92-104. Epub 2010 Jan 8. PMID: 20067571

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