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

CI Nussbaum-Krammer, MF Neto… - JoVE (Journal of …, 2015 - jove.com
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 …