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Molecular and Cellular Biology Volume 21, 2001 - Issue 7
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Cell Growth and Development

A Protease-Resistant 61-Residue Prion Peptide Causes Neurodegeneration in Transgenic Mice

Surachai Supattapone1 Institute for Neurodegenerative Diseases;2 Departments of Neurology
,
Essia Bouzamondo3 Pathology
,
Haydn L. Ball1 Institute for Neurodegenerative Diseases;2 Departments of Neurology
,
Holger Wille1 Institute for Neurodegenerative Diseases;2 Departments of Neurology
,
Hoang-Oanh B. Nguyen1 Institute for Neurodegenerative Diseases;2 Departments of Neurology
,
Fred E. Cohen1 Institute for Neurodegenerative Diseases;4 Cellular and Molecular Pharmacology;5 Medicine;6 Biochemistry and Biophysics, University of California at San Francisco, San Francisco, California94143
,
Stephen J. DeArmond1 Institute for Neurodegenerative Diseases;3 Pathology
,
Stanley B. Prusiner1 Institute for Neurodegenerative Diseases;2 Departments of Neurology;6 Biochemistry and Biophysics, University of California at San Francisco, San Francisco, California94143
&
Michael Scott1 Institute for Neurodegenerative Diseases;2 Departments of Neurology
 show all
Pages 2608-2616 | Received 29 Sep 2000, Accepted 18 Dec 2000, Published online: 27 Mar 2023

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Jesús R Requena & Holger Wille. (2014) The structure of the infectious prion protein. Prion 8:1, pages 60-66.
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Chin Jung Cheng & Valerie Daggett. (2014) Different misfolding mechanisms converge on common conformational changes. Prion 8:1, pages 125-135.
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Articles from other publishers (36)

Priyanka Majumder & Oishee Chakrabarti. (2017) Lysosomal Quality Control in Prion Diseases. Molecular Neurobiology 55:3, pages 2631-2644.
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Biswanath Chatterjee, Chung-Yu Lee, Chen Lin, Eric H.-L. Chen, Chao-Li Huang, Chien-Chih Yang & Rita P.-Y. Chen. (2013) Amyloid Core Formed of Full-Length Recombinant Mouse Prion Protein Involves Sequence 127–143 but Not Sequence 107–126. PLoS ONE 8:7, pages e67967.
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Yuzuru Taguchi, Arla M. A. Mistica, Tetsuyuki Kitamoto & Hermann M. Schätzl. (2013) Critical Significance of the Region between Helix 1 and 2 for Efficient Dominant-Negative Inhibition by Conversion-Incompetent Prion Protein. PLoS Pathogens 9:6, pages e1003466.
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Shirley Hesketh, Andrew R. Thompsett & David R. Brown. (2012) Prion protein polymerisation triggered by manganese-generated prion protein seeds. Journal of Neurochemistry 120:1, pages 177-189.
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Tai-Yan Liao, Lily Y.-L. Lee & Rita P.-Y. Chen. (2011) Leu138 in bovine prion peptide fibrils is involved in seeding discrimination related to codon 129 M/V polymorphism in the prion peptide seeding experiment. FEBS Journal 278:22, pages 4351-4361.
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Marc W. van der Kamp & Valerie Daggett. 2011. Prion Proteins. Prion Proteins 169 197 .
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Marc W. van der Kamp & Valerie Daggett. (2009) The consequences of pathogenic mutations to the human prion protein. Protein Engineering, Design and Selection 22:8, pages 461-468.
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Christopher J. Silva. 2009. Sequelae and Long-Term Consequences of Infectious Diseases. Sequelae and Long-Term Consequences of Infectious Diseases 425 442 .
Giorgio Colombo, Massimiliano Meli, Giulia Morra, Ruth Gabizon & María Gasset. (2009) Methionine Sulfoxides on Prion Protein Helix-3 Switch on the α-Fold Destabilization Required for Conversion. PLoS ONE 4:1, pages e4296.
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Aarthi Ashok & Ramanujan S. Hegde. (2008) Retrotranslocation of Prion Proteins from the Endoplasmic Reticulum by Preventing GPI Signal Transamidation. Molecular Biology of the Cell 19:8, pages 3463-3476.
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Adriano AguzziFrank BaumannJuliane Bremer. (2008) The Prion's Elusive Reason for Being. Annual Review of Neuroscience 31:1, pages 439-477.
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Peter M. H. Heegaard, Ulrik Boas & Daniel E. Otzen. (2007) Dendrimer Effects on Peptide and Protein Fibrillation. Macromolecular Bioscience 7:8, pages 1047-1059.
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Lily Y.-L. Lee & Rita P.-Y. Chen. (2007) Quantifying the Sequence-Dependent Species Barrier between Hamster and Mouse Prions. Journal of the American Chemical Society 129:6, pages 1644-1652.
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Ghanashyam D. Ghadge, Lijun Wang, Kamal Sharma, Anna Liza Monti, Vytas Bindokas, Fred J. Stevens & Raymond P. Roos. (2006) Truncated wild-type SOD1 and FALS-linked mutant SOD1 cause neural cell death in the chick embryo spinal cord. Neurobiology of Disease 21:1, pages 194-205.
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A. Grosset, K. Moskowitz, C. Nelsen, T. Pan, E. Davidson & C.S. Orser. (2005) Rapid presymptomatic detection of PrPSc via conformationally responsive palindromic PrP peptides. Peptides 26:11, pages 2193-2200.
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Rodrigo Morales, Claudio Hetz & Claudio Soto. 2005. Neurodegeneration and Prion Disease. Neurodegeneration and Prion Disease 319 344 .
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Katarina M. Luhr, Elin K. Nordström, Peter Löw, Hans-Gustaf Ljunggren, Albert Taraboulos & Krister Kristensson. (2004) Scrapie Protein Degradation by Cysteine Proteases in CD11c + Dendritic Cells and GT1-1 Neuronal Cells . Journal of Virology 78:9, pages 4776-4782.
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Malin K Sandberg, Peter Wallén, Martin A Wikström & Krister Kristensson. (2004) Scrapie-infected GT1-1 cells show impaired function of voltage-gated N-type calcium channels (Cav 2.2) which is ameliorated by quinacrine treatment. Neurobiology of Disease 15:1, pages 143-151.
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Peter V. Farrelly, Bronwyn L. Kenna, Karina L. Laohachai, Randa Bahadi, Mario Salmona, Gianluigi Forloni & Joseph I. Kourie. (2003) Quinacrine blocks PrP (106-126)-formed channels. Journal of Neuroscience Research 74:6, pages 934-941.
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David Bolton. 2003. Prions and Mad Cow Disease. Prions and Mad Cow Disease.
Randa Bahadi, Peter V. Farrelly, Bronwyn L. Kenna, Joseph I. Kourie, Fabrizio Tagliavini, Gianluigi Forloni & Mario Salmona. (2003) Channels formed with a mutant prion protein PrP(82-146) homologous to a 7-kDa fragment in diseased brain of GSS patients. American Journal of Physiology-Cell Physiology 285:4, pages C862-C872.
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Wen-Quan Zou, Sabina Capellari, Piero Parchi, Man-Sun Sy, Pierluigi Gambetti & Shu G. Chen. (2003) Identification of Novel Proteinase K-resistant C-terminal Fragments of PrP in Creutzfeldt-Jakob Disease. Journal of Biological Chemistry 278:42, pages 40429-40436.
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Roberto Chiesa, Pedro Piccardo, Elena Quaglio, Bettina Drisaldi, San Ling Si-Hoe, Masaki Takao, Bernardino Ghetti & David A. Harris. (2003) Molecular Distinction between Pathogenic and Infectious Properties of the Prion Protein. Journal of Virology 77:13, pages 7611-7622.
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G.P. Concepcion & E.A. Padlan. (2003) Are humans getting ‘mad-cow disease’ from eating beef, or something else?. Medical Hypotheses 60:5, pages 699-701.
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Carsten Korth, Kiyotoshi Kaneko, Darlene Groth, Norbert Heye, Glenn Telling, James Mastrianni, Piero Parchi, Pierluigi Gambetti, Robert Will, James Ironside, Cornelia Heinrich, Patrick Tremblay, Stephen J. DeArmond & Stanley B. Prusiner. (2003) Abbreviated incubation times for human prions in mice expressing a chimeric mouse–human prion protein transgene. Proceedings of the National Academy of Sciences 100:8, pages 4784-4789.
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Stephen J DeArmond & Stanley B Prusiner. (2003) Perspectives on prion biology, prion disease pathogenesis, and pharmacologic approaches to treatment. Clinics in Laboratory Medicine 23:1, pages 1-41.
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Anne Bellon & Martin Vey. 2002. Structure-Function Relationships of Human Pathogenic Viruses. Structure-Function Relationships of Human Pathogenic Viruses 255 294 .
H.L. Ball, D.S. King, F.E. Cohen, S.B. Prusiner & M.A. Baldwin. (2001) Engineering the prion protein using chemical synthesis. The Journal of Peptide Research 58:5, pages 357-374.
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Roberto Chiesa & David A. Harris. (2001) Prion Diseases: What Is the Neurotoxic Molecule?. Neurobiology of Disease 8:5, pages 743-763.
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Surachai Supattapone, Holger Wille, Lisa Uyechi, Jiri Safar, Patrick Tremblay, Francis C. Szoka, Fred E. Cohen, Stanley B. Prusiner & Michael R. Scott. (2001) Branched Polyamines Cure Prion-Infected Neuroblastoma Cells. Journal of Virology 75:7, pages 3453-3461.
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