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A naturally occurring variant of the human prion protein completely prevents prion disease

Author

Listed:
  • Emmanuel A. Asante

    (MRC Prion Unit, UCL Institute of Neurology)

  • Michelle Smidak

    (MRC Prion Unit, UCL Institute of Neurology)

  • Andrew Grimshaw

    (MRC Prion Unit, UCL Institute of Neurology)

  • Richard Houghton

    (MRC Prion Unit, UCL Institute of Neurology
    † Present address: Strangeways Research Laboratory, Worts’ Causeway, Cambridge CB8 8UX, UK)

  • Andrew Tomlinson

    (MRC Prion Unit, UCL Institute of Neurology)

  • Asif Jeelani

    (MRC Prion Unit, UCL Institute of Neurology)

  • Tatiana Jakubcova

    (MRC Prion Unit, UCL Institute of Neurology)

  • Shyma Hamdan

    (MRC Prion Unit, UCL Institute of Neurology)

  • Angela Richard-Londt

    (MRC Prion Unit, UCL Institute of Neurology)

  • Jacqueline M. Linehan

    (MRC Prion Unit, UCL Institute of Neurology)

  • Sebastian Brandner

    (MRC Prion Unit, UCL Institute of Neurology)

  • Michael Alpers

    (MRC Prion Unit, UCL Institute of Neurology
    Papua New Guinea Institute of Medical Research, Goroka)

  • Jerome Whitfield

    (MRC Prion Unit, UCL Institute of Neurology
    Papua New Guinea Institute of Medical Research, Goroka)

  • Simon Mead

    (MRC Prion Unit, UCL Institute of Neurology)

  • Jonathan D. F. Wadsworth

    (MRC Prion Unit, UCL Institute of Neurology)

  • John Collinge

    (MRC Prion Unit, UCL Institute of Neurology)

Abstract

This study looks at a polymorphism of the human prion protein gene, which results in a G-to-V substitution at residue 127, in transgenic mice expressing different human prion proteins, finding that mice heterozygous for the G127V polymorphism are resistant to both kuru and classical CJD prions, but there is some transmission of variant CJD prions; most remarkable, however, is that mice homozygous for V127 are completely resistant to all prion strains.

Suggested Citation

  • Emmanuel A. Asante & Michelle Smidak & Andrew Grimshaw & Richard Houghton & Andrew Tomlinson & Asif Jeelani & Tatiana Jakubcova & Shyma Hamdan & Angela Richard-Londt & Jacqueline M. Linehan & Sebastia, 2015. "A naturally occurring variant of the human prion protein completely prevents prion disease," Nature, Nature, vol. 522(7557), pages 478-481, June.
    • Handle: RePEc:nat:nature:v:522:y:2015:i:7557:d:10.1038_nature14510
    DOI: 10.1038/nature14510
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