Prion-like disorders: blurring the divide between transmissibility and infectivity

摘要: Prions are proteins that access self-templating amyloid forms, which confer phenotypic changes can spread from individual to within or between species. These infectious phenotypes be beneficial, as with yeast prions, deleterious, mammalian prions transmit spongiform encephalopathies. However, the ability form is not unique prion proteins. Diverse polypeptides tend populate intrinsically unfolded states also conformers associated devastating neurodegenerative disorders. Moreover, two RNA-binding proteins, FUS and TDP-43, cytoplasmic aggregates in amyotrophic lateral sclerosis, harbor a ‘prion domain’ similar those found several Can these diseases they linked become ‘infectious’ too? Here, we highlight advances define transmissibility of forms connected Alzheimer's disease, Parkinson's disease Huntington's disease. Collectively, findings suggest cell brains afflicted individuals, thereby spreading specific distinctive protein being converted amyloid. Importantly, this mandates re-evaluation emerging neuronal graft stem-cell therapies. In Commentary, how treatments might optimized overcome transmissible neurodegeneration.