From Prion Diseases to Prion-Like Propagation Mechanisms of Neurodegenerative Diseases
Joint Authors
Imberdis, Thibaut
Acquatella-Tran Van Ba, Isabelle
Perrier, Véronique
Source
International Journal of Cell Biology
Issue
Vol. 2013, Issue 2013 (31 Dec. 2013), pp.1-8, 8 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2013-10-10
Country of Publication
Egypt
No. of Pages
8
Main Subjects
Abstract EN
Prion diseases are fatal neurodegenerative sporadic, inherited, or acquired disorders.
In humans, Creutzfeldt-Jakob disease is the most studied prion disease.
In animals, the most frequent prion diseases are scrapie in sheep and goat, bovine spongiform encephalopathy in cattle, and the emerging chronic wasting disease in wild and captive deer in North America.
The hallmark of prion diseases is the deposition in the brain of PrPSc, an abnormal β-sheet-rich form of the cellular prion protein (PrPC) (Prusiner 1982).
According to the prion hypothesis, PrPSc can trigger the autocatalytic conversion of PrPC into PrPSc, presumably in the presence of cofactors (lipids and small RNAs) that have been recently identified.
In this review, we will come back to the original works that led to the discovery of prions and to the protein-only hypothesis proposed by Dr.
Prusiner.
We will then describe the recent reports on mammalian synthetic prions and recombinant prions that strongly support the protein-only hypothesis.
The new concept of “deformed templating” regarding a new mechanism of PrPSc formation and replication will be exposed.
The review will end with a chapter on the prion-like propagation of other neurodegenerative disorders, such as Alzheimer’s and Parkinson’s disease and tauopathies.
American Psychological Association (APA)
Acquatella-Tran Van Ba, Isabelle& Imberdis, Thibaut& Perrier, Véronique. 2013. From Prion Diseases to Prion-Like Propagation Mechanisms of Neurodegenerative Diseases. International Journal of Cell Biology،Vol. 2013, no. 2013, pp.1-8.
https://search.emarefa.net/detail/BIM-512882
Modern Language Association (MLA)
Acquatella-Tran Van Ba, Isabelle…[et al.]. From Prion Diseases to Prion-Like Propagation Mechanisms of Neurodegenerative Diseases. International Journal of Cell Biology No. 2013 (2013), pp.1-8.
https://search.emarefa.net/detail/BIM-512882
American Medical Association (AMA)
Acquatella-Tran Van Ba, Isabelle& Imberdis, Thibaut& Perrier, Véronique. From Prion Diseases to Prion-Like Propagation Mechanisms of Neurodegenerative Diseases. International Journal of Cell Biology. 2013. Vol. 2013, no. 2013, pp.1-8.
https://search.emarefa.net/detail/BIM-512882
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-512882