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

Emmanuel A. Asante, Michelle Smidak, Andrew Grimshaw, Richard Houghton, Andrew Tomlinson, Asif Jeelani, Tatiana Jakubcova, Shyma Hamdan, Angela Richard-Londt, Jacqueline M. Linehan, Sebastian Brandner, Michael Alpers, Jerome Whitfield, Simon Mead, Jonathan D. F. Wadsworth and John Collinge ()
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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
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
Jerome Whitfield: MRC Prion Unit, UCL Institute of Neurology
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

Nature, 2015, vol. 522, issue 7557, 478-481

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.

Date: 2015
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DOI: 10.1038/nature14510

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