Abstract
Aberrant protein folding underpins many neurodegenerative diseases as well as certain myopathies and cancers. Protein misfolding can be driven by the presence of distinctive prion and prion-like regions within certain proteins. These prion and prion-like regions have also been found to drive liquid-liquid phase separation. Liquid-liquid phase separation is thought to be an important physiological process, but one that is prone to malfunction. Thus, aberrant liquid-to-solid phase transitions may drive protein aggregation and fibrillization, which could give rise to pathological inclusions. Here, we review prions and prion-like proteins, their roles in phase separation and disease, as well as potential therapeutic approaches to counter aberrant phase transitions.
Original language | English |
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Article number | 1014 |
Journal | Biomolecules |
Volume | 11 |
Issue number | 7 |
DOIs | |
State | Published - Jul 2021 |
Keywords
- Aberrant phase transitions
- Amyloid
- Chaperones
- Liquid-liquid phase separation
- Prion-like domains
- Prions
- Protein misfolding