Modeling the cell biology of prions

Richard Rubenstein, Robert B. Petersen

Research output: Chapter in Book/Report/Conference proceedingChapterpeer-review

Abstract

Cell models have been useful for elucidating the function of proteins and/or theirrole in pathogenesis. Even before the discovery that the prion protein was a normal cellular protein (Oesch et al. Cell 40 (4):735–746, 1985), cell models were developed to investigate prion infection (Rubenstein et al. J Gen Virol 65 (Pt 12):2191–2198, 1984). Subsequently, withthe discovery of familial forms of human prion diseases (Hsiao et al. Nature 338 (6213):342–345, 1989), cell models were developed to investigate the effect of mutations on the metabolism of the prion protein and, in parallel, the normal synthesis and processing of the cellular prion protein. In this chapter, we review the progress made in these two areas to date.

Original languageEnglish
Title of host publicationPrions and Diseases
Subtitle of host publicationVolume 1, Physiology and Pathophysiology
PublisherSpringer New York
Pages181-192
Number of pages12
ISBN (Electronic)9781461453055
ISBN (Print)9781461453048
DOIs
StatePublished - Jan 1 2013

Keywords

  • Cell models
  • Cellular cultures
  • Pathogenic mutations
  • Prion protein
  • Prions
  • TSE agent replication

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