Prion Diseases pp 269-301 | Cite as

Transgenic Mouse Models of Prion Diseases

  • Julie Moreno
  • Glenn C. TellingEmail author
Part of the Neuromethods book series (NM, volume 129)


Seminal studies showed that optimal disease progression requires matching of the primary structures of PrPSc and PrPC paved the way for the development of Tg models in which to study human prions and subsequently other naturally occurring mammalian prions. Here we review how transgenic mouse models are being used to elucidate basic molecular mechanisms of prion propagation and strain variation, and to address the likelihood of interspecies prion transmissions, in particular the zoonotic potential of various animal prion strains.

Key words

Quasispecies Species barriers Strains Transgenic models 


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Copyright information

© Springer Science+Business Media LLC 2017

Authors and Affiliations

  1. 1.Department of Microbiology, Immunology and Pathology, Cell and Molecular Biology Graduate Program, Molecular, Cellular and Integrative Neuroscience Graduate ProgramPrion Research Center, Colorado State UniversityFort CollinsUSA

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