Prions, prion-like prionoids, and neurodegenerative disordersVacancy

• Published in: Annals of the Indian Academy of Neurology 2016-04, Vol.19 (2), p.169-174
• Main Author: Verma, Ashok
• Format: Article
• Language: English
• Subjects: Alzheimer's disease; Amyotrophic lateral sclerosis; Brain research; Creutzfeldt-Jakob disease; Disease transmission; Medical research; Mutation; Neurodegeneration; prion; prionoid; Prions; Propagation; protein aggregate; Proteins; Researchers
• ISSN: 0972-2327; 1998-3549
• DOI: 10.4103/0972-2327.179979

Prion diseases or transmissible spongiform encephalopathies are fatal neurodegenerative diseases characterized by the aggregation and deposition of the misfolded prion protein in the brain. α-synuclein (α-syn)-associated multiple system atrophy has been recently shown to be caused by a bona fide α-syn prion strain. Several other misfolded native proteins such as β-amyloid, tau and TDP-43 share some aspects of prions although none of them is shown to be transmissible in nature or in experimental animals. However, these prion-like "prionoids" are causal to a variety of neurodegenerative diseases such as Alzheimer's disease, Parkinson's disease, and amyotrophic lateral sclerosis. The remarkable recent discovery of at least two new α-syn prion strains and their transmissibility in transgenic mice and in vitro cell models raises a distinct question as to whether some specific strain of other prionoids could have the capability of disease transmission in a manner similar to prions. In this overview, we briefly describe human and other mammalian prion diseases and comment on certain similarities between prion and prionoid and the possibility of prion-like transmissibility of some prionoid strains.