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Widening Spectrum of Prions Causing Neurodegenerative Diseases

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Proteopathic Seeds and Neurodegenerative Diseases

Part of the book series: Research and Perspectives in Alzheimer's Disease ((ALZHEIMER))

Abstract

The field of prion biology still seems to be in its infancy. Over the past three decades, there has been a steady accumulation of evidence that each neurodegenerative disease is caused by a particular protein that becomes a prion. As with the prion diseases caused by the aberrant prion protein (PrPSc), amyloid deposits in other neurodegenerative disorders were found to have the same protein as that identified by molecular genetic studies of patients with inherited neurodegeneration. While the number of prions identified in mammals (now at more than half a dozen) and in fungi (now more than ten) will undoubtedly continue to expand, we have no idea about prions in all the other phylogeny. The mammalian prions composed of PrP, Aβ, tau, α-synuclein, SOD1 and huntingtin all cause distinct neurodegenerative diseases. In each of these disorders, the respective mammalian proteins adopt a β-sheet–rich conformation that readily oligomerizes and becomes self-propagating. The oligomeric states of mammalian prions are thought to be the toxic forms, and assembly into larger polymers such as amyloid fibrils seems to be a mechanism for minimizing toxicity. To date, there is not a single medication that halts or even slows a neurodegenerative disease caused by prions. This may be a bellwether of the unique pathogenic mechanisms that feature in each of the prion diseases and of the urgent need to develop informative molecular diagnostics and effective antiprion therapeutics.

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  1. Institute for Neurodegenerative Diseases, University of California, 675 Nelson Rising Ln, Room 318, 0518, San Francisco, CA, 94143, USA

    Stanley B. Prusiner

  2. Department of Neurology, University of California, San Francisco, CA, 94143, USA

    Stanley B. Prusiner

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  1. Stanley B. Prusiner
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  1. , Dept. Cellular Neurology, University of Tübingen, Otfried-Mueller-Str. 27, Tübingen, 72076, Germany

    Mathias Jucker

  2. Fondation IPSEN, Quai Georges Gorse 65, Boulogne-Billancourt Cedex, 92650, France

    Yves Christen

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Prusiner, S.B. (2013). Widening Spectrum of Prions Causing Neurodegenerative Diseases. In: Jucker, M., Christen, Y. (eds) Proteopathic Seeds and Neurodegenerative Diseases. Research and Perspectives in Alzheimer's Disease. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-35491-5_1

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