Chronic wasting disease prion infection of differentiated neurospheres

Prion. 2017 Jul 4;11(4):277-283. doi: 10.1080/19336896.2017.1336273. Epub 2017 Aug 1.

Abstract

A possible strategy to develop more diverse cell culture systems permissive to infection with naturally occurring prions is to exploit culture of neurospheres from transgenic mice expressing the normal prion protein (PrP) of the native host species. Accordingly, we developed differentiated neurosphere cultures from the cervid PrP-expressing mice to investigate whether this in vitro system would support replication of non-adapted cervid-origin chronic wasting disease (CWD) prions. Here we report the successful amplification of disease-associated PrP in differentiated neurosphere cultures within 3 weeks after exposure to CWD prions from both white-tailed deer or elk. This neurosphere culture system provides a new in vitro tool that can be used to assess non-adapted CWD prion propagation and transmission.

Keywords: Chronic wasting disease; Neurosphere; Prion; Transgenic mice.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Brain / metabolism
  • Brain / pathology*
  • Cell Differentiation
  • Deer
  • Disease Models, Animal
  • Gene Expression Regulation
  • Mice
  • Mice, Transgenic
  • Neurons / metabolism
  • Neurons / pathology*
  • PrPC Proteins / metabolism*
  • Prions / physiology*
  • Wasting Disease, Chronic / etiology
  • Wasting Disease, Chronic / pathology
  • Wasting Disease, Chronic / transmission*

Substances

  • PrPC Proteins
  • Prions