Phasing Biology Reveals the Role of Prion Protein

Abstract

Prion is a misfolded protein that has a unique propagation system by the change of the tertiary structure. The concept of prion is conserved among various species, such as bacteria, yeast, anaspidea, sheep, and human. A kind of prions, such as mammalian PrP, causes a serious disease by widespread neuronal dysfunction. However, it remains unclear why the hallmark concept of prions has been preserved across diverse species form prokaryotes to eukaryotes. Here we summarize the researches of prion proteins about the function of the cellular prion or the dysfunction triggering neurotoxicity. We also highlight key findings that liquid-liquid phase separation (LLPS) of proteins plays a crucial role for the existence of prion proteins in living organisms. The LLPS of protein is expected as a new research target for polymer science, solution thermodynamics, biophysics, spectroscopy, and drug discovery.