Abstract:
:Prion diseases are transmissible neurodegenerative disorders that include scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle and Creutzfeldt-Jakob disease (CJD) in humans. The principal component of the infectious agent responsible for these diseases appears to be an abnormal isoform of the host-encoded prion protein (PrP), designated PrP(Sc). Prion diseases are transmissible to the same or different mammalian species by inoculation with, or dietary exposure to, infected tissues. Although scrapie in sheep has been recognized for over 200 years, it is the recent epidemic of BSE that has centred much public and scientific attention on these neurodegenerative diseases. The occurrence of variant CJD in humans and the experimental confirmation that it is caused by the same prion strain as BSE has highlighted the need for intensive study into the pathogenesis of these diseases and new diagnostic and therapeutic approaches. The existence and implications of subclinical forms of prion disease are discussed.
journal_name
Trends Microbioljournal_title
Trends in microbiologyauthors
Hill AF,Collinge Jdoi
10.1016/j.tim.2003.10.007subject
Has Abstractpub_date
2003-12-01 00:00:00pages
578-84issue
12eissn
0966-842Xissn
1878-4380pii
S0966842X03002877journal_volume
11pub_type
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