Inherited Prion Disease A117V Is Not Simply a Proteinopathy but Produces Prions Transmissible to Transgenic Mice Expressing Homologous Prion Protein

• Emmanuel A. Asante,

  Affiliation Medical Research Council Prion Unit and Department of Neurodegenerative Disease, University College London Institute of Neurology, Queen Square, London, United Kingdom

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• Jacqueline M. Linehan,

  Affiliation Medical Research Council Prion Unit and Department of Neurodegenerative Disease, University College London Institute of Neurology, Queen Square, London, United Kingdom

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• Michelle Smidak,

  Affiliation Medical Research Council Prion Unit and Department of Neurodegenerative Disease, University College London Institute of Neurology, Queen Square, London, United Kingdom

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• Andrew Tomlinson,

  Affiliation Medical Research Council Prion Unit and Department of Neurodegenerative Disease, University College London Institute of Neurology, Queen Square, London, United Kingdom

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• Andrew Grimshaw,

  Affiliation Medical Research Council Prion Unit and Department of Neurodegenerative Disease, University College London Institute of Neurology, Queen Square, London, United Kingdom

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• Asif Jeelani,

  Affiliation Medical Research Council Prion Unit and Department of Neurodegenerative Disease, University College London Institute of Neurology, Queen Square, London, United Kingdom

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• Tatiana Jakubcova,

  Affiliation Medical Research Council Prion Unit and Department of Neurodegenerative Disease, University College London Institute of Neurology, Queen Square, London, United Kingdom

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• Shyma Hamdan,

  Affiliation Medical Research Council Prion Unit and Department of Neurodegenerative Disease, University College London Institute of Neurology, Queen Square, London, United Kingdom

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• Caroline Powell,

  Affiliation Medical Research Council Prion Unit and Department of Neurodegenerative Disease, University College London Institute of Neurology, Queen Square, London, United Kingdom

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• Sebastian Brandner,

  Affiliation Medical Research Council Prion Unit and Department of Neurodegenerative Disease, University College London Institute of Neurology, Queen Square, London, United Kingdom

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• Jonathan D. F. Wadsworth,

  Affiliation Medical Research Council Prion Unit and Department of Neurodegenerative Disease, University College London Institute of Neurology, Queen Square, London, United Kingdom

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• John Collinge

  * E-mail: j.collinge@prion.ucl.ac.uk

  Affiliation Medical Research Council Prion Unit and Department of Neurodegenerative Disease, University College London Institute of Neurology, Queen Square, London, United Kingdom

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