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Transgenic Mouse Models in Prion Transmission Studies

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Abstract

Prion diseases are a unique group of mostly transmissible ­neurodegenerative diseases where the ubiquitous cellular prion protein (PrPC) plays a central role. Numerous transgenic mouse models have been instrumental in dissecting the roles of PrPC and other factors in the replication, pathogenesis, and transmission of the prion agents. This chapter summarizes the seminal roles of transgenic mouse models in prion transmission studies with an emphasis on the contributions of PrP primary sequence and prion strains to prion transmission barriers and non-PrP factors in prion pathogenesis.

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  1. Department of Pathology, Case Western Reserve University, 11100 Euclid Ave, Cleveland, OH, 44106, USA

    Qingzhong Kong Ph.D.

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Correspondence to Qingzhong Kong Ph.D. .

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  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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Kong, Q. (2013). Transgenic Mouse Models in Prion Transmission Studies. In: Zou, WQ., Gambetti, P. (eds) Prions and Diseases. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-5338-3_11

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