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Prions and Diseases pp 193–204Cite as

Prion Strain Interference

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Abstract

Prions are transmissible agents that comprised of a misfolded protein PrPSc that is posttranslationally derived from the normal isoform PrPC. Prion strains are operationally defined by differences in the distribution and intensity of spongiform degeneration and distribution of PrPSc in the CNS. The mechanism by which prion strains are encoded is not known; however, current evidence suggests that the conformation of PrPSc encodes prion strain diversity. In natural prion disease, more than one prion strain can exist in an individual. Prion strains, when present in the same host, can interfere with each other, a process that may be important during prion adaptation following interspecies transmission. While the parameters that influence prion strain interference are beginning to be described, the mechanism responsible for strain interference is not known.

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Acknowledgments

This work was supported by the National Center for Research Resources (P20 RR0115635-6 and C06 RR17417-01) and the National Institute for Neurological Disorders and Stroke (R01 NS052609).

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Authors and Affiliations

  1. Department of Medical Microbiology and Immunology, School of Medicine, Creighton University, Omaha, NE, 68178, USA

    Charles R. Schutt Ph.D, Ronald A. Shikiya Ph.D & Jason C. Bartz Ph.D.

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  1. Charles R. Schutt Ph.D
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  2. Ronald A. Shikiya Ph.D
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  3. Jason C. Bartz Ph.D.
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Correspondence to Jason C. Bartz Ph.D. .

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Editors and Affiliations

  1. , Department of Pathology, Case Western Reserve University School o, 2085 Adelbert Road, Cleveland, 44106, Ohio, USA

    Wen-Quan Zou

  2. , Department of Neuropathology, Case Western Reserve University School o, 2085 Adelbert Road, Cleveland, 44106, Ohio, USA

    Pierluigi Gambetti

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Schutt, C.R., Shikiya, R.A., Bartz, J.C. (2013). Prion Strain Interference. In: Zou, WQ., Gambetti, P. (eds) Prions and Diseases. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-5305-5_14

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