Caenorhabditis elegans as a model organism for protein homeostasis diseases

Age-associated protein homeostasis diseases, such as Parkinson’s or Alzheimer’s disease, are caused by the misfolding and aggregation of proteins, disrupting essential cellular processes and resulting in cell death. Throughout life, cellular health is surveyed by the highly conserved proteostasis network (PN), comprising molecular chaperones and components facilitating degradation and clearance of misfolded or aggregated proteins. Cell culture and mouse model studies have aided significantly to our understanding of the molecular pathogenesis of protein homeostasis diseases. However, the complexity of these diseases requires a versatile model system that combines the ease of cell culture with the complexity of an entire organism while possessing high conservation of components of the PN. Such a model system is the nematode Caenorhabditis elegans. This chapter will focus on the human protein misfolding diseases that have been modeled in C. elegans.