Prions, prion-like prionoids, and neurodegenerative disordersVacancy

Research output: Contribution to journalReview article

3 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)169-174
Number of pages6
JournalAnnals of Indian Academy of Neurology
Volume19
Issue number2
DOIs
StatePublished - Apr 1 2016

Keywords

  • Neurodegeneration
  • prion
  • prionoid
  • protein aggregate

ASJC Scopus subject areas

  • Clinical Neurology

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