Transgenic Overexpression of the Disordered Prion Protein N1 Fragment in Mice Does Not Protect Against Neurodegenerative Diseases Due to Impaired ER Translocation

[1]  Y. Cordeiro,et al.  RNA modulates aggregation of the recombinant mammalian prion protein by direct interaction , 2019, Scientific Reports.

[2]  R. Vandenberghe,et al.  Aβ-induced acceleration of Alzheimer-related τ-pathology spreading and its association with prion protein , 2019, Acta Neuropathologica.

[3]  M. Frosch,et al.  PrP-grafted antibodies bind certain amyloid β-protein aggregates, but do not prevent toxicity , 2019, Brain Research.

[4]  D. Harris,et al.  Prion neurotoxicity , 2019, Brain pathology.

[5]  C. Sigurdson,et al.  Cellular and Molecular Mechanisms of Prion Disease. , 2019, Annual review of pathology.

[6]  R. Zimmermann,et al.  The signal peptide plus a cluster of positive charges in prion protein dictate chaperone-mediated Sec61 channel gating , 2019, Biology Open.

[7]  S. Strittmatter,et al.  Liquid and Hydrogel Phases of PrPC Linked to Conformation Shifts and Triggered by Alzheimer's Amyloid-β Oligomers. , 2018, Molecular cell.

[8]  M. Rowan,et al.  Cellular Prion Protein Mediates the Disruption of Hippocampal Synaptic Plasticity by Soluble Tau In Vivo , 2018, The Journal of Neuroscience.

[9]  D. Harris,et al.  Prions activate a p38 MAPK synaptotoxic signaling pathway , 2018, PLoS pathogens.

[10]  D. Willbold,et al.  A d-enantiomeric peptide interferes with heteroassociation of amyloid-β oligomers and prion protein , 2018, The Journal of Biological Chemistry.

[11]  W. Surewicz,et al.  Identification of prion protein-derived peptides of potential use in Alzheimer's disease therapy. , 2018, Biochimica et biophysica acta. Molecular basis of disease.

[12]  Markus Glatzel,et al.  Alterations in the brain interactome of the intrinsically disordered N-terminal domain of the cellular prion protein (PrPC) in Alzheimer’s disease , 2018, PloS one.

[13]  Katrine Bugge,et al.  Extreme disorder in an ultrahigh-affinity protein complex , 2018, Nature.

[14]  Jessica Siltberg-Liberles,et al.  Interaction of Peptide Aptamers with Prion Protein Central Domain Promotes α-Cleavage of PrPC , 2018, Molecular Neurobiology.

[15]  P. Saftig,et al.  Diverse functions of the prion protein - Does proteolytic processing hold the key? , 2017, Biochimica et biophysica acta. Molecular cell research.

[16]  H. Koch,et al.  The Sec61/SecY complex is inherently deficient in translocating intrinsically disordered proteins , 2017, The Journal of Biological Chemistry.

[17]  I. Zerr,et al.  α-synuclein interacts with PrPC to induce cognitive impairment through mGluR5 and NMDAR2B , 2017, Nature Neuroscience.

[18]  David W. Colby,et al.  The N-terminus of the prion protein is a toxic effector regulated by the C-terminus , 2017, eLife.

[19]  W. Surewicz,et al.  Amyloid fibrils from the N-terminal prion protein fragment are infectious , 2016, Proceedings of the National Academy of Sciences.

[20]  S. Hornemann,et al.  The prion protein is an agonistic ligand of the G protein-coupled receptor Adgrg6 , 2016, Nature.

[21]  W. Surewicz,et al.  Soluble prion protein and its N-terminal fragment prevent impairment of synaptic plasticity by Aβ oligomers: Implications for novel therapeutic strategy in Alzheimer's disease , 2016, Neurobiology of Disease.

[22]  D. Harris,et al.  A Neuronal Culture System to Detect Prion Synaptotoxicity , 2016, PLoS pathogens.

[23]  B. Puig,et al.  Secretory pathway retention of mutant prion protein induces p38-MAPK activation and lethal disease in mice , 2016, Scientific Reports.

[24]  C. Betzel,et al.  Exosomal cellular prion protein drives fibrillization of amyloid beta and counteracts amyloid beta‐mediated neurotoxicity , 2016, Journal of neurochemistry.

[25]  S. Strittmatter,et al.  Metabotropic glutamate receptor 5 couples cellular prion protein to intracellular signalling in Alzheimer's disease. , 2016, Brain : a journal of neurology.

[26]  N. Hooper,et al.  Prion protein “gamma-cleavage”: characterizing a novel endoproteolytic processing event , 2016, Cellular and Molecular Life Sciences.

[27]  J. Lucas,et al.  Prion-mediated neurodegeneration is associated with early impairment of the ubiquitin–proteasome system , 2015, Acta Neuropathologica.

[28]  H. Nieznańska,et al.  Stabilization of microtubular cytoskeleton protects neurons from toxicity of N-terminal fragment of cytosolic prion protein. , 2015, Biochimica et biophysica acta.

[29]  P. Peters,et al.  Subcellular distribution of the prion protein in sickness and in health. , 2015, Virus research.

[30]  S. Tabrizi,et al.  Prion degradation pathways: Potential for therapeutic intervention , 2015, Molecular and Cellular Neuroscience.

[31]  P. Liberski,et al.  The sheddase ADAM10 is a potent modulator of prion disease , 2015, eLife.

[32]  Eric W. Danielson,et al.  SynPAnal: Software for Rapid Quantification of the Density and Intensity of Protein Puncta from Fluorescence Microscopy Images of Neurons , 2014, PloS one.

[33]  Ű. Langel,et al.  Targeting prion propagation using peptide constructs with signal sequence motifs. , 2014, Archives of biochemistry and biophysics.

[34]  X. Roucou,et al.  Aβ induces its own prion protein N-terminal fragment (PrPN1)–mediated neutralization in amorphous aggregates , 2014, Neurobiology of Aging.

[35]  R. Bujdoso,et al.  Cytosolic PrP Can Participate in Prion-Mediated Toxicity , 2014, Journal of Virology.

[36]  H. Schlüter,et al.  High molecular mass assemblies of amyloid-β oligomers bind prion protein in patients with Alzheimer's disease. , 2014, Brain : a journal of neurology.

[37]  X. Roucou,et al.  Taking advantage of physiological proteolytic processing of the prion protein for a therapeutic perspective in prion and Alzheimer diseases , 2014, Prion.

[38]  Deming Zhao,et al.  Metabolism of minor isoforms of prion proteins: Cytosolic prion protein and transmembrane prion protein , 2013, Neural regeneration research.

[39]  David A. Scott,et al.  Genome engineering using the CRISPR-Cas9 system , 2013, Nature Protocols.

[40]  A. Vortmeyer,et al.  Metabotropic Glutamate Receptor 5 Is a Coreceptor for Alzheimer Aβ Oligomer Bound to Cellular Prion Protein , 2013, Neuron.

[41]  B. Puig,et al.  Roles of endoproteolytic α‐cleavage and shedding of the prion protein in neurodegeneration , 2013, The FEBS journal.

[42]  Tiziana Sonati,et al.  The toxicity of antiprion antibodies is mediated by the flexible tail of the prion protein , 2013, Nature.

[43]  K. Büssow,et al.  High level transient production of recombinant antibodies and antibody fusion proteins in HEK293 cells , 2013, BMC Biotechnology.

[44]  N. Hunter,et al.  The PrP(C) C1 fragment derived from the ovine A136R154R171PRNP allele is highly abundant in sheep brain and inhibits fibrillisation of full-length PrP(C) protein in vitro. , 2013, Biochimica et biophysica acta.

[45]  Guy Nimrod,et al.  The α-Helical Structure of Prodomains Promotes Translocation of Intrinsically Disordered Neuropeptide Hormones into the Endoplasmic Reticulum* , 2013, The Journal of Biological Chemistry.

[46]  J. Luebke,et al.  A Mutant Prion Protein Sensitizes Neurons to Glutamate-Induced Excitotoxicity , 2013, The Journal of Neuroscience.

[47]  N. Hooper,et al.  Prion Protein-mediated Toxicity of Amyloid-β Oligomers Requires Lipid Rafts and the Transmembrane LRP1* , 2013, The Journal of Biological Chemistry.

[48]  M. Gobbi,et al.  An N-terminal Fragment of the Prion Protein Binds to Amyloid-β Oligomers and Inhibits Their Neurotoxicity in Vivo* , 2013, The Journal of Biological Chemistry.

[49]  D. Bennett,et al.  The Complex PrPc-Fyn Couples Human Oligomeric Aβ with Pathological Tau Changes in Alzheimer's Disease , 2012, The Journal of Neuroscience.

[50]  Y. Sugita‐Konishi,et al.  Cellular Prion Protein: From Physiology to Pathology , 2012, Viruses.

[51]  Qingzhong Kong,et al.  α-Cleavage of cellular prion protein , 2012, Prion.

[52]  W. Surewicz,et al.  Soluble Prion Protein Inhibits Amyloid-β (Aβ) Fibrillization and Toxicity* , 2012, The Journal of Biological Chemistry.

[53]  A. Vortmeyer,et al.  Alzheimer Amyloid-β Oligomer Bound to Post-Synaptic Prion Protein Activates Fyn to Impair Neurons , 2012, Nature Neuroscience.

[54]  P. Saá,et al.  The N-Terminal, Polybasic Region of PrPC Dictates the Efficiency of Prion Propagation by Binding to PrPSc , 2012, The Journal of Neuroscience.

[55]  D. Harris,et al.  Prion protein at the crossroads of physiology and disease , 2012, Trends in Neurosciences.

[56]  N. Hooper,et al.  Prion protein facilitates uptake of zinc into neuronal cells , 2012, Nature Communications.

[57]  I. Solomon,et al.  Ion channels induced by the prion protein , 2012, Prion.

[58]  F. Checler,et al.  α-Secretase-derived Fragment of Cellular Prion, N1, Protects against Monomeric and Oligomeric Amyloid β (Aβ)-associated Cell Death* , 2011, The Journal of Biological Chemistry.

[59]  X. Roucou,et al.  The prion protein unstructured N‐terminal region is a broad‐spectrum molecular sensor with diverse and contrasting potential functions , 2011, Journal of neurochemistry.

[60]  D. Harris,et al.  A Naturally Occurring C-terminal Fragment of the Prion Protein (PrP) Delays Disease and Acts as a Dominant-negative Inhibitor of PrPSc Formation* , 2011, The Journal of Biological Chemistry.

[61]  A. Goldberg,et al.  Misfolded PrP impairs the UPS by interaction with the 20S proteasome and inhibition of substrate entry , 2011, The EMBO journal.

[62]  S. Lindquist,et al.  The cellular prion protein mediates neurotoxic signalling of β‐sheet‐rich conformers independent of prion replication , 2011, The EMBO journal.

[63]  H. Orzechowski,et al.  The Extracellular Regulated Kinase-1 (ERK1) Controls Regulated α-Secretase-mediated Processing, Promoter Transactivation, and mRNA Levels of the Cellular Prion Protein* , 2011, The Journal of Biological Chemistry.

[64]  I. Solomon,et al.  An N-terminal Polybasic Domain and Cell Surface Localization Are Required for Mutant Prion Protein Toxicity* , 2011, The Journal of Biological Chemistry.

[65]  R. Hegde,et al.  Compartment-Restricted Biotinylation Reveals Novel Features of Prion Protein Metabolism in Vivo , 2010, Molecular biology of the cell.

[66]  G. Forloni,et al.  Cell Type-Specific Neuroprotective Activity of Untranslocated Prion Protein , 2010, PloS one.

[67]  A. Aguzzi,et al.  Supplementary figure legends , 2010 .

[68]  W. Surewicz,et al.  Interaction between human prion protein and amyloid-beta (Abeta) oligomers: role OF N-terminal residues. , 2010, The Journal of biological chemistry.

[69]  Chunjiang Yu,et al.  Endocytic pathways mediating oligomeric Aβ42 neurotoxicity , 2010, Molecular Neurodegeneration.

[70]  A. Aguzzi,et al.  Unexpected Tolerance of α-Cleavage of the Prion Protein to Sequence Variations , 2010, PloS one.

[71]  F. Checler,et al.  The α-Secretase-derived N-terminal Product of Cellular Prion, N1, Displays Neuroprotective Function in Vitro and in Vivo* , 2009, The Journal of Biological Chemistry.

[72]  C. Masters,et al.  Increased Proportions of C1 Truncated Prion Protein Protect Against Cellular M1000 Prion Infection , 2009, Journal of neuropathology and experimental neurology.

[73]  H. Laude,et al.  Proteasome inhibitors promote the sequestration of PrPSc into aggresomes within the cytosol of prion-infected CAD neuronal cells. , 2009, The Journal of general virology.

[74]  K. Winklhofer,et al.  α-Helical Domains Promote Translocation of Intrinsically Disordered Polypeptides into the Endoplasmic Reticulum* , 2009, The Journal of Biological Chemistry.

[75]  R. Hegde,et al.  Functional Depletion of Mahogunin by Cytosolically Exposed Prion Protein Contributes to Neurodegeneration , 2009, Cell.

[76]  R. Hegde,et al.  Selective Processing and Metabolism of Disease-Causing Mutant Prion Proteins , 2009, PLoS pathogens.

[77]  Stephanie L. K. Bowers,et al.  Cytoplasmic prion protein induces forebrain neurotoxicity. , 2009, Biochimica et biophysica acta.

[78]  R. Hegde,et al.  Prion protein biosynthesis and its emerging role in neurodegeneration. , 2009, Trends in biochemical sciences.

[79]  G. Zamponi Faculty Opinions recommendation of Cellular prion protein mediates impairment of synaptic plasticity by amyloid-beta oligomers. , 2009 .

[80]  John W. Gilbert,et al.  Cellular Prion Protein Mediates Impairment of Synaptic Plasticity by Amyloid-β Oligomers , 2009, Nature.

[81]  N. Hooper,et al.  α-cleavage of the prion protein occurs in a late compartment of the secretory pathway and is independent of lipid rafts , 2009, Molecular and Cellular Neuroscience.

[82]  R. Hegde,et al.  Reduced translocation of nascent prion protein during ER stress contributes to neurodegeneration. , 2008, Developmental cell.

[83]  N. Ben-Tal,et al.  Stress‐protective signalling of prion protein is corrupted by scrapie prions , 2008, The EMBO journal.

[84]  M. Almeida,et al.  Prion Protein Complexed to N2a Cellular RNAs through Its N-terminal Domain Forms Aggregates and Is Toxic to Murine Neuroblastoma Cells*S⃞ , 2008, Journal of Biological Chemistry.

[85]  K. Bedecs,et al.  Antiprion properties of prion protein‐derived cell‐penetrating peptides , 2008, FASEB journal : official publication of the Federation of American Societies for Experimental Biology.

[86]  S. Moestrup,et al.  LRP1 controls biosynthetic and endocytic trafficking of neuronal prion protein , 2008, Journal of Cell Science.

[87]  J. Collinge,et al.  Disease-associated prion protein oligomers inhibit the 26S proteasome. , 2007, Molecular cell.

[88]  R. Williamson,et al.  Toward Molecular Dissection of PrPC-PrPSc Interactions* , 2007, Journal of Biological Chemistry.

[89]  N. Hooper,et al.  The low-density lipoprotein receptor-related protein 1 (LRP1) mediates the endocytosis of the cellular prion protein. , 2007, The Biochemical journal.

[90]  K. Roth,et al.  N-Terminally Deleted Forms of the Prion Protein Activate Both Bax-Dependent and Bax-Independent Neurotoxic Pathways , 2007, The Journal of Neuroscience.

[91]  A. Aguzzi,et al.  Lethal recessive myelin toxicity of prion protein lacking its central domain , 2007, The EMBO journal.

[92]  S. Kaech,et al.  Culturing hippocampal neurons , 2006, Nature Protocols.

[93]  E. Walter,et al.  The affinity of copper binding to the prion protein octarepeat domain: evidence for negative cooperativity. , 2006, Biochemistry.

[94]  D. Rubinsztein,et al.  The roles of intracellular protein-degradation pathways in neurodegeneration , 2006, Nature.

[95]  R. Chiesa,et al.  Conditions of Endoplasmic Reticulum Stress Favor the Accumulation of Cytosolic Prion Protein* , 2006, Journal of Biological Chemistry.

[96]  R. Hegde,et al.  The surprising complexity of signal sequences. , 2006, Trends in biochemical sciences.

[97]  X. Roucou,et al.  Molecular morphology and toxicity of cytoplasmic prion protein aggregates in neuronal and non‐neuronal cells , 2006, Journal of neurochemistry.

[98]  L. Arterburn,et al.  The Interaction between Cytoplasmic Prion Protein and the Hydrophobic Lipid Core of Membrane Correlates with Neurotoxicity* , 2006, Journal of Biological Chemistry.

[99]  J. Collinge,et al.  Disease-related Prion Protein Forms Aggresomes in Neuronal Cells Leading to Caspase Activation and Apoptosis* , 2005, Journal of Biological Chemistry.

[100]  N. Hooper,et al.  Assigning functions to distinct regions of the N-terminus of the prion protein that are involved in its copper-stimulated, clathrin-dependent endocytosis , 2005, Journal of Cell Science.

[101]  A. Pramanik,et al.  Membrane perturbation effects of peptides derived from the N-termini of unprocessed prion proteins. , 2005, Biochimica et biophysica acta.

[102]  G. J. Raymond,et al.  The most infectious prion protein particles , 2005, Nature.

[103]  R. S. Stewart,et al.  Cytosolic Prion Protein (PrP) Is Not Toxic in N2a Cells and Primary Neurons Expressing Pathogenic PrP Mutations* , 2005, Journal of Biological Chemistry.

[104]  T. Onodera,et al.  Alpha‐ and beta‐ cleavages of the amino‐terminus of the cellular prion protein , 2004, Biology of the cell.

[105]  K. Winklhofer,et al.  The C-terminal Globular Domain of the Prion Protein Is Necessary and Sufficient for Import into the Endoplasmic Reticulum* , 2004, Journal of Biological Chemistry.

[106]  R. S. Stewart,et al.  Mutational Analysis of Topological Determinants in Prion Protein (PrP) and Measurement of Transmembrane and Cytosolic PrP during Prion Infection* , 2003, Journal of Biological Chemistry.

[107]  Sebastian Brandner,et al.  Depleting Neuronal PrP in Prion Infection Prevents Disease and Reverses Spongiosis , 2003, Science.

[108]  X. Roucou,et al.  Cytosolic Prion Protein Is Not Toxic and Protects against Bax-mediated Cell Death in Human Primary Neurons* , 2003, Journal of Biological Chemistry.

[109]  N. Hooper,et al.  The N-terminal Region of the Prion Protein Ectodomain Contains a Lipid Raft Targeting Determinant* , 2003, Journal of Biological Chemistry.

[110]  S. Prusiner,et al.  Cytosolic Prion Protein in Neurons , 2003, The Journal of Neuroscience.

[111]  R. S. Stewart,et al.  Mutant PrP Is Delayed in Its Exit from the Endoplasmic Reticulum, but Neither Wild-type nor Mutant PrP Undergoes Retrotranslocation Prior to Proteasomal Degradation* , 2003, Journal of Biological Chemistry.

[112]  H. Schätzl,et al.  Essential Role of the Prion Protein N Terminus in Subcellular Trafficking and Half-life of Cellular Prion Protein* , 2003, The Journal of Biological Chemistry.

[113]  Ű. Langel,et al.  Cell membrane translocation of the N-terminal (1-28) part of the prion protein. , 2002, Biochemical and biophysical research communications.

[114]  S. Lindquist,et al.  Neurotoxicity and Neurodegeneration When PrP Accumulates in the Cytosol , 2002, Science.

[115]  S. Lindquist,et al.  Wild-type PrP and a mutant associated with prion disease are subject to retrograde transport and proteasome degradation , 2001, Proceedings of the National Academy of Sciences of the United States of America.

[116]  Thomas D. Schmittgen,et al.  Analysis of relative gene expression data using real-time quantitative PCR and the 2(-Delta Delta C(T)) Method. , 2001, Methods.

[117]  Albert Taraboulos,et al.  Proteasomes and ubiquitin are involved in the turnover of the wild‐type prion protein , 2001, The EMBO journal.

[118]  F. Cohen,et al.  Identification of Two Prion Protein Regions That Modify Scrapie Incubation Time , 2001, Journal of Virology.

[119]  C. V. Mering,et al.  Prion Protein Devoid of the Octapeptide Repeat Region Restores Susceptibility to Scrapie in PrP Knockout Mice , 2000, Neuron.

[120]  K Wüthrich,et al.  NMR solution structure of the human prion protein. , 2000, Proceedings of the National Academy of Sciences of the United States of America.

[121]  Christian von Mering,et al.  Expression of Amino-Terminally Truncated PrP in the Mouse Leading to Ataxia and Specific Cerebellar Lesions , 1998, Cell.

[122]  P E Wright,et al.  Structure of the recombinant full-length hamster prion protein PrP(29-231): the N terminus is highly flexible. , 1997, Proceedings of the National Academy of Sciences of the United States of America.

[123]  K Wüthrich,et al.  NMR characterization of the full‐length recombinant murine prion protein, mPrP(23–231) , 1997, FEBS letters.

[124]  R. Riek,et al.  NMR structure of the mouse prion protein domain PrP(121–231) , 1996, Nature.

[125]  A. Aguzzi,et al.  Prion protein (PrP) with amino‐proximal deletions restoring susceptibility of PrP knockout mice to scrapie. , 1996, The EMBO journal.

[126]  D. Teplow,et al.  Truncated Forms of the Human Prion Protein in Normal Brain and in Prion Diseases (*) , 1995, The Journal of Biological Chemistry.

[127]  K. Moulder,et al.  The N-terminal Domain of a Glycolipid-anchored Prion Protein Is Essential for Its Endocytosis via Clathrin-coated Pits (*) , 1995, The Journal of Biological Chemistry.

[128]  P. Lansbury,et al.  Cell-free formation of protease-resistant prion protein , 1994, Nature.

[129]  D. Harris,et al.  A prion protein cycles between the cell surface and an endocytic compartment in cultured neuroblastoma cells. , 1993, The Journal of biological chemistry.

[130]  A. Aguzzi,et al.  Mice devoid of PrP are resistant to scrapie , 1993, Cell.

[131]  B. Chait,et al.  Processing of a cellular prion protein: identification of N- and C-terminal cleavage sites. , 1993, Biochemistry.

[132]  K. Winklhofer,et al.  Targeting of the prion protein to the cytosol: mechanisms and consequences. , 2010, Current issues in molecular biology.