Prion-like proteins in phase separation and their link to disease

Macy L. Sprunger, Meredith E. Jackrel

Research output: Contribution to journalReview articlepeer-review

24 Scopus citations

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 languageEnglish
Article number1014
JournalBiomolecules
Volume11
Issue number7
DOIs
StatePublished - Jul 2021

Keywords

  • Aberrant phase transitions
  • Amyloid
  • Chaperones
  • Liquid-liquid phase separation
  • Prion-like domains
  • Prions
  • Protein misfolding

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