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The pathogenic mechanisms of prion diseases

Authors
 Kwon Hur  ;  Jae Il Kim  ;  Seung Il Choi  ;  Eun Kyoung Choi  ;  Richard I Carp  ;  Yong Sun Kim 
Citation
 MECHANISMS OF AGEING AND DEVELOPMENT, Vol.123(12) : 1637-1647, 2002 
Journal Title
 MECHANISMS OF AGEING AND DEVELOPMENT 
ISSN
 0047-6374 
Issue Date
2002
MeSH
Animals ; Humans ; Oxidative Stress/physiology ; Prion Diseases/etiology* ; Prion Diseases/metabolism* ; Prion Diseases/physiopathology ; Prions/metabolism*
Keywords
Transmissible spongiform encephalopathies (TSEs) or prion diseases ; Prion protein (PrPC, PrPSc) ; Pathogenic mechanisms ; Neurodegeneration ; Oxidative stress
Abstract
Transmissible spongiform encephalopathies (TSEs) or prion diseases are a group of fatal neurodegenerative diseases of humans and animals, including bovine spongiform encephalopathy (BSE) of cattle, scrapie of sheep, and Creutzfeldt–Jakob disease (CJD) of humans. Prion diseases have become an important issue in public health and in the scientific world not only due to the possible relationship between BSE and new variant CJD (nvCJD) but also due to the unique biological features of the infectious agent. Although the nature of the infectious agent and the pathogenic mechanisms of prion diseases are not fully understood, considerable evidence suggests that an abnormal form (PrPSc) of a host prion protein (PrPC) may compose substantial parts of the infectious agent and that various factors such as oxidative stress and calcium cytotoxicity are associated with the pathogenesis of prion diseases. Here, we briefly review and discuss the pathogenic mechanisms of prion diseases. These advances in understandings of fundamental biology of prion diseases may open the possibilities for the prevention and treatment of these unusual diseases and also suggest applications in more common neurodegenerative disorders such as Alzheimer's disease (AD) and Parkinson's disease (PD).
Full Text
http://www.sciencedirect.com/science/article/pii/S0047637402000994
DOI
10.1016/S0047-6374(02)00099-4
Appears in Collections:
5. Research Institutes (연구소) > Corneal Dystrophy Research Institute (각막이상증연구소) > 1. Journal Papers
Yonsei Authors
Choi, Seung Il(최승일) ORCID logo https://orcid.org/0000-0001-7168-8795
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/143968
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