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Prions, prion-like prionoids, and neurodegenerative disorders #MMPMID27293325
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Ann Indian Acad Neurol 2016[Apr]; 19 (2): 169-74 PMID27293325show ga
Prion diseases or transmissible spongiform encephalopathies are fatal neurodegenerative diseases characterized by the aggregation and deposition of the misfolded prion protein in the brain. ?-synuclein (?-syn)-associated multiple system atrophy has been recently shown to be caused by a bona fide ?-syn prion strain. Several other misfolded native proteins such as ?-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 ?-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.