Official List of "Priority" Papers
  1. Regulation of GABAA and Glutamate Receptor Expression, Synaptic Facilitation and Long-Term Potentiation in the Hippocampus of Prion Mutant Mice. Rangel et al. PLoS One, 2009, 4(10): e7592.
  2. Complementarity determining regions of a funtional anti-PrP scFv orchestrate conformation-specificity and antiprion activity. Muller- Schiffmann et al. Mol. Immunol., 2009, 46: 532-40.
  3. NMR structure of the human prion protein with the pathological Q212P mutation reveals unique structural features. Ilc et al. PLoS One, 2010, 5(7):e11715.
  4. Characterization of the role of dendritic cells in prion transfer to primary neurons. Langevin et al. Biochem. J, 2010, 431:189-98.
  5. Structural facets of disease-linked human prion protein mutants: a molecular dynamic study. Rossetti et al. Proteins, 2010, 78: 3270-80.
  6. Combining independent drug classes into superior, synergistically acting hybrid molecules. Müller-Schiffmann et al. Angew. Chem. Int. Ed., 2010, 49:8743-46.
  7. Prion infection of differentiated neurospheres. Hervá et al. J. Neurosci. Methods, 2010, 188:270-275.
  8. Toward the Molecular Basis of Inherited Prion Diseases: NMR Structure of the Human Prion Protein with V210I Mutation. Biljan et al. J. Mol. Biol., 2011, 412:660–673.
  9. De novo prions. Benetti et al. Biol Rep., 2010, 2: 46-50.
  10. Neurodevelopmental expression and localization of the cellular prion protein in the central nervous system of the mouse.                Benvegnù et al.               J. Comp. Neurol., 2010, 518:1879-91.
  11. NMR structure of the human prion protein with the pathological Q212P mutation reveals unique structural features. Ilc et al. PLoS One., 2010, 5(7):e11715.
  12. Combining independent drug classes into superior, synergistically acting hybrid molecules. Müller-Schiffmann et al. Angewandte Chemie Int. Edition, 2010, 49:8743-46.
  13. Pharmacological PrP-Silencing Accelerates CNS Autoimmune Disease via TCR Signaling. Hu et al. Brain, 2010, 133: 375-388.
  14. Oral treatment with the amyloid-b oligomer precipitating substance D3 improves pathology of Alzheimer’s transgenic mice. Funke et al. ACS Chemical Neuroscience, 2010, 1: 639-48.
  15. Structure-activity relationship of tocopherol derivatives suggesting a novel non-antioxidant mechanism in antiprion potency. Muyrers et al. Neurosci. Letters, 2010, 469:122-6.
  16. Quinpramine - a promising compound for treating immune-mediated demyelination of the nervous system. Meyer zur Hörste et al. Drug News & Perspectives, 2010, 23:287-94.
  17. Common Structural Traits across Pathogenic Mutants of the Human Prion Protein and Their Implications for Familial Prion Diseases. Rosetti et al. J. Mol. Biol., 2011, 411:700-712.
  18. Probing structural differences between PrPC and PrPSc by surface nitration and acetylation: evidence of conformational change in the C-terminus. Gong et al. Biochemistry, 2011, 50: 4963-72.
  19. Aerosols transmit prions to immunocompetent and immunodeficient mice. Haybaeck et al. PLoS Pathog., 2011, 7(1):e1001257.
  20. Prion disease blood test using immunoprecipitation and improved quaking-induced conversion. Orrú et al. MBio., 2011, 2(3) pii: e00078-11.
  21. Biological Effects and Use of PrPSc- and PrP-Specific Antibodies Generated by Immunization with Purified Full-Length Native Mouse Prions. Petsch et al. J. Virol., 2011, 4538–4546.
  22. Quinpramine ameliorates rat experimental autoimmune neuritis and redistributes MHC class II molecules. Meyer zu Hörste et al. PLoS One, 2011, 6: 6:e21223.
  23. Prion uptake in the gut: identification of the first uptake and replication sites. Kujala et al. PloS Pathog., 2011, 7(12): e1002449.
  24. Biological Effects and Use of PrPSc- and PrP-Specific Antibodies Generated by Immunization with Purified Full-Length Native Mouse Prions. Petsch et al. J. Virol., 2011, 85: 4538–4546.
  25. Neuroprotective role of PrPC against kainate-induced epileptic seizures and cell death depends on the modulation of JNK3 activation by GluR6/7–PSD-95 binding. Carulla et al. Mol. Biol. Cell, 2011, 22: 3041–3054.
  26. Atypical/Nor98 scrapie infectivity in sheep peripheral tissues. Andreoletti et al. PLoS Pathog., 2011, 7: e1001285.
  27. A system-level approach for deciphering the transcriptional response to prion infection. Zampieri et al. Bioinformatics, 2011, 27: 3407-14.
  28. Biological effects and use of antibodies generated by immunizing with full length native prions. Petsch et al. J. Virol., 2011, 85: 4538-46.
  29. Quinpramine ameliorates rat experimental autoimmune neuritis and redistributes MHC class II molecules. Meyer zu Hörste et al. PLoS ONE, 2011, 6(6):e21223.
  30. Genetic polymorphism at codon 129 of the prion protein gene is not associated with primary progressive multiple sclerosis. Stüve et al. Arch. Neurol., 2011, 68: 264-265.
  31. Presenilin-1 (PSEN1) but not amyloid precursor protein (APP) mutations present in mouse modles of Alzheimer's disease attenuate the response to γ-secretase modulators (GSMs) regardless of their potency and structure. Hahn et al. J. Neurochem., 2011, 116: 385-95.
  32. Rapidly progressive Alzheimer's disease. Schmidt et al. Arch. Neurol., 2011, 68:1124-30.
  33. Structural Organization of Mammalian Prions as Probed by Limited Proteolysis. Vázquez-Fernández, E. et al. PLoS One, 2012, 7(11): e50111.
  34. PK-sensitive PrPSc Is Infectious and Shares Basic Structural Features with PK-resistant PrPSc. Sajnani et al. PLoS Pathog., 2012, 8(3): e1002547.
  35. Multifaceted roles of tunneling nanotubes in intercellular communication. Marzo et al. Front. Physiol., 2012, 3:72.
  36. Prionemia and leukocyte-platelet-associated infectivity in sheep transmissible spongiform encephalopathy models. Lacroux et al. J. Virol., 2012, 86: 2056–2066.
  37. Role of the cellular prion protein in oligodendrocyte precursor cell proliferation and differentiation in the developing and adult mouse CNS. Bribian et al. PLoS One, 2012, 7(4): e33872.
  38. Persistence of the bovine spongiform encephalopathy infectious agent in sewage. Maluquer de Motes et al. Environ. Res., 2012, 117: 1-7.
  39. Prion Pathogenesis Is Faithfully Reproduced in Cerebellar Organotypic Slice Cultures. Falsig et al. PLoS Pathog., 2012, 8(11): e1002985.
  40. Prion propagation, toxicity and degradation. Aguzzi & Falsig. Nat. Neurosci., 2012, 15: 936-939.
  41. Five Questions on Prion Diseases. Aguzzi & Zhu. PLoS Pathog., 2012, 8(5): e1002651.
  42. Determining the role of mononuclear phagocytes in prion neuroinvasion from the skin. Wathne et al. J. Leukoc. Biol., 2012, 91:817-28.
  43. The Effects of Host Age on the Transport of Complement-Bound Complexes to the Spleen and the Pathogenesis of Intravenous Scrapie Infection. Brown et al. J. Virol., 2012, 86: 25–35.
  44. PK-sensitive PrPSc Is Infectious and Shares Basic Structural Features with PK-resistant PrPSc. Sajnani et al. PLoS Pathog., 2012, 8(3): e1002547.
  45. Sporadic Creutzfeldt–Jakob disease subtype-specific alterations of the brain proteome: Impact on Rab3a recycling. Gawinecka et al. Proteomics, 2012,        12: 3610-20.
  46. Impact of Leucocyte Depletion and Prion Reduction Filters on TSE Blood Borne Transmission. Lacroux et al. PLoS One, 2012, 7(7): e42019.
  47. Highly Efficient Prion Transmission by Blood Transfusion. Andreoletti et al. PLoS Pathog., 2012, 8(6): e1002782.
  48. Structural Rearrangements at Physiological pH: Nuclear Magnetic Resonance Insights from the V210I Human Prion Protein Mutant. Biljan et al. Biochemistry, 2012, 51: 7465−7474.
  49. Structural basis for the protective effect of the human prion protein carrying the dominant-negative E219K polymorphism. Biljan et al. Biochem. J., 2012, 446: 243–251.
  50. Early structural features in mammalian prion conformation conversion.              Legname. Prion, 2012, 6: 37-39.
  51. Aggregated proteins in chronic mental diseases: DISC1opathies. Korth. Prion, 6:1-8.
  52. Human and rat brain lipofuscin proteome. Ottis et al. Proteomics, 2012, 15-16:2445-54.
  53. C-terminal fragment of N-cadherin accelerates synapse destabilization by β-amyloid. Andreyeva et al. Brain, 2012, 135: 2140-54.
  54. Cellular prion protein participates in amyloid-beta transcytosis across the blood-brain barrier. Pflanzner et al. Blood Flow and Metabolism, 2012, 32: 628-33.
  55. Hybrid compounds - from simple combinations to nanomachines. Müller-Schiffmann et al. BioDrugs, 2012, 26:21-31.
  56. Prions, proteinase K and infectivity. Sajnani & Requena. Prion, 2012, 6:430-2
  57. Report about four novel mutations in the prion protein gene. Schelzke et al. Dement Geriatr. Cogn. Disord., 2013, 35: 229-37.
  58. Subtype-specific synaptic proteome alterations in sporadic Creutzfeldt-Jakob disease. Gawinecka et al. J. Alzheimers Dis., 2013, 37: 51-61.
  59. Neurotoxicity of prion peptides mimicking the central domain of the cellular prion protein. Vilches et al. PLoS One, 2013, 8(8): e70881.
  60. Cellular prion protein modulates beta-amyloid deposition in aged APP/PS1 transgenic mice. Ordonez-Gutierrez et al.           Neurobiol. Aging, 2013, 34:2793-804.
  61. Microarray and deep sequencing cross-platform analysis of the mirRNome and isomiR variation in response to epidermal growth factor. Llorens et al. BMC Gennomics, 2013, 14: 371-5.
  62. PrP(C) regulates epidermal growth factor receptor function and cell shape dynamics in Neuro2a cells.               Llorens et al. J Neurochem., 2013, 127: 124-138.
  63. A highly expressed miR-101 isomiR is a functional silencing small RNA.Llorens et al. BMC Genomics, 2013, 14: 104-107.
  64. Prion formation correlates with activation of translation-regulating protein 4E-BP and neuronal transcription factor Elk1. Allard et al.              Neurobiol. Dis., 2013, 58: 116-122.
  65. Prion strings and networks on the surface of infected cells. Rouvinski et al. J. Cell Biol., 2013, 204: 423-41.
  66. Description of a novel viral tool to identify and quantify ovine faecal pollution in the environment. Rusiñol et al. Sci. Total Environ., 2013, 1:458-460
  67. Dominant-negative effects in prion diseases: insights from molecular dynamics simulations on mouse prion protein chimeras. Cong et al. J. Biomol. Struct. Dyn., 2013, 31: 829-40.
  68. Probing early misfolding events in prion protein mutants by NMR spectroscopy. Giachin et al. Molecules, 2013, 18: 9451-76.
  69. Prion protein accumulation in lipid rafts of mouse aging brain. Agostini et al. PLoS One., 2013, 8:e74244.
  70. SAXS structural study of PrPSc reveals ~11 nm diameter of basic double intertwined fibers. Amenitsch et al.   Prion, 2013, 7:496-500.
  71. Exploring the role of lipids in intercellular conduits: breakthroughs in the pipeline. Delage & Zurzolo. Frontiers in Plant Science, 2013, 4: 504.
  72. The Fate of PrP GPI-Anchor Signal Peptide is Modulated by P238S Pathogenic Mutation. Guizzunti & Zurzolo. Traffic, 2013, 15: 78-93.
  73. Not on the menu: autophagy-independent clearance of prions. Browman & Zurzolo. Prion, 2013, 286-90.
  74. Myo10 is a key regulator of TNT formation in neuronal cells. Gousset et al. J. Cell Sci., 2013, 126: 4424-35.
  75. Pathogenic prions deviate PrP(C) signaling in neuronal cells and impair A-beta clearance. Pradines et al. Cell Death and Dis., 2013, 4:e456.
  76. Spontaneous Generation of Infectious Prion Disease in Transgenic Mice. Torres et al. Emerging Infect. Dis., 2013, 19: 1938-47.
  77. Bovine spongiform encephalopathy induces misfolding of alleged prion-resistant species cellular prion protein without altering its pathobiological features. Vidal et al. J. Neurosci., 2013, 33: 7778-86.
  78. Cellular prion protein modulates β-amyloid deposition in aged APP/PS1 transgenic mice. Ordóñez-Gutiérrez et al. Neurobiol Aging, 2013, 34:2793-804.
  79. PrP(C) regulates epidermal growth factor receptor function and cell shape dynamics in Neuro2a cells..               Llorens et al. J. Neurochem., 2013, 127:               124-38.
  80. A template for new drugs against Alzheimer's Disease. Aguzzi et al. Cell, 2013, 154:1182‐1184.
  81. The immunobiology of prion diseases. Aguzzi et al. Nature Reviews. Immunology, 2013, 13: 888‐902.
  82. Mutations in the gene encoding PDGF‐B cause brain calcifications in humans and mice. Keller et al. Nature Genetics, 2013, 45:1077‐1082.
  83. SIRPα polymorphisms, but not the prion protein, control phagocytosis of apoptotic cells. Nuvolone et al. J. Exp. Med., 2013, 210:2539‐2552.
  84. The toxicity of antiprion antibodies is mediated by the flexible tail of the prion protein. Sonati et al. Nature, 2013, 501:102‐106.
  85. Aging-induced proteostatic changes in the rat hippocampus identify ARP3, NEB2 and BRAG2 as a molecular circuitry for cognitive impairment. Ottis et al. PLoS ONE, 2013, 8: e75112.
  86. Characterization of a single-chain variable fragment recognizing a linear epitope of Abeta: a biotechnological tool for studies on Alzheimer's disease? Dornieden et al. PLoS ONE, 2013, 8(3):e59820.
  87. Hybrid molecules synergistically acting against protein aggregation diseases. Korth et al. Curr. Top. Med. Chem., 2013, 13: 2484-90.
  88. Oxidation of methionine 216 in sheep and elk prion protein is highly dependent upon the amino acid at position 218 but is not important for prion propagation. Silva et al. Biochemistry, 2013, 52: 2139-47.
  89. Association of prion protein genotype and scrapie prion protein type with cellular prion protein charge isoform profiles in cerebrospinal fluid of humans with sporadic or familial prion diseases.           Schmitz et al. Neurobiol. Aging, 2014, 35: 1177-88.
  90. Impact of the cellular prion protein on amyloid-β and 3PO-tau processing. Schmitz et al. J. Alzheimers Dis., 2014, 38: 551-65.
  91. Anchorless 23-230 PrPC Interactomics for Elucidation of PrPC Protective Role. Zafar et al. Mol. Neurobiol., 2014, 49:1385-99.
  92. Detection of infectivity in blood of persons with variant and sporadic Creutzfeldt-Jakob disease. Douet et al. Emerg. Infect. Dis., 2014, 20: 114-7.
  93. Glycogen synthase kinase 3 beta (GSK3beta) at the tip of neuronal development and regeneration. Seira and Del Rio. Mol. Neurobiol., 2014,         49: 931-44.
  94. Gene expression resulting from PrP(C) ablation and PrP (C) overexpression in murine and cellular models. Llorens et al. Mol. Neurobiol., 2014, 49: 413-23.
  95. In vitro aggregation assays for the characterization of α-synuclein prion-like properties. Narkiewicz et al. Prion, 2014, 8:34-56.
  96. Accelerated clinical course of prion disease in mice compromised in repair of oxidative DNA damage. Jalland et al. Free Radic. Biol. Med., 2014, 68: 1-7.
  97. Golgi sorting regulates organization and activity of GPI proteins at apical membranes. Paladino et al.   Nature Chem. Biol., 2014, 10: 350-7.
  98. Detection of infectivity in the blood of vCJD and sCJD affected patients. Douet et al. Emerging Infect. Dis., 2014, 20:114-7.
  99. Evidence of sub-clinical prion disease in aged mice following exposure to bovine spongiform encephalopathy. Brown & Mabbott. J. Gen. Virol., 2014, 95: 231-243.
  100. The structure of the infectious prion protein: Experimental data and molecular models. Requena & Wille. Prion, 2014, 8:60-6.
  101. Evidence for zoonotic potential of ovine scrapie prions. Cassard et al. Nature Commun., 2014, 5:5821.
  102. Loss of prion protein leads to age-dependent behavioral abnormalities and changes in cytoskeletal protein expression. Schmitz et al. Mol. Neurobiol., 2014, 50:923-36.
  103. Aging and the mucosal immune system in the intestine. Mabbott et al. Biogerontology, 2015, 13:133-45.

Characteristic CSF prion seeding efficiency in humans with prion diseases. Cramm et al. Mol. Neurobiol., 2015, 51:396-405.

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