Investigating the spreading and toxicity of prion-like proteins using the metazoan model organism C. Elegans

Carmen I. Nussbaum-Krammer*, Mário F. Neto, Renée M. Brielmann, Jesper S. Pedersen, Richard I. Morimoto

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

131 Scopus citations


Prions are unconventional self-propagating proteinaceous particles, devoid of any coding nucleic acid. These proteinaceous seeds serve as templates for the conversion and replication of their benign cellular isoform. Accumulating evidence suggests that many protein aggregates can act as self-propagating templates and corrupt the folding of cognate proteins. Although aggregates can be functional under certain circumstances, this process often leads to the disruption of the cellular protein homeostasis (proteostasis), eventually leading to devastating diseases such as Alzheimer’s disease (AD), Parkinson’s disease (PD), Amyotrophic lateral sclerosis (ALS), or transmissible spongiform encephalopathies (TSEs). The exact mechanisms of prion propagation and cell-to-cell spreading of protein aggregates are still subjects of intense investigation. To further this knowledge, recently a new metazoan model in Caenorhabditis elegans, for expression of the prion domain of the cytosolic yeast prion protein Sup35 has been established. This prion model offers several advantages, as it allows direct monitoring of the fluorescently tagged prion domain in living animals and ease of genetic approaches. Described here are methods to study prion-like behavior of protein aggregates and to identify modifiers of prion-induced toxicity using C. Elegans.

Original languageEnglish (US)
Article numbere52321
JournalJournal of Visualized Experiments
Issue number95
StatePublished - Jan 8 2015


  • Caenorhabditis elegans
  • Cell-to-cell transmission
  • Cellular Biology
  • Issue 95
  • Neurodegenerative diseases
  • Non-cell autonomous toxicity
  • Prion-like spreading
  • Protein aggregation
  • Protein misfolding diseases
  • Proteostasis

ASJC Scopus subject areas

  • General Chemical Engineering
  • General Biochemistry, Genetics and Molecular Biology
  • General Immunology and Microbiology
  • General Neuroscience


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