Abstract:
:Prion diseases are transmissible neurodegenerative conditions that include Creutzfeldt-Jakob disease (CJD) in humans and bovine spongiform encephalopathy (BSE) and scrapie in animals. Prions appear to be composed principally or entirely of abnormal isoforms of a host-encoded glycoprotein, prion protein. Prion propagation involves recruitment of host cellular prion protein, composed primarily of alpha-helical structure, into a disease specific isoform rich in beta-sheet structure. The existence of multiple prion strains has been difficult to explain in terms of a protein-only infections agent, but recent studies suggest that strain specific phenotypes can be encoded by different prion protein conformations and glycosylation patterns. The ability of a protein to encode phenotypic information has important biological implications. The appearance of a novel human prion disease, variant CJD, and the clear experimental evidence that it is caused by exposure to BSE has highlighted the need to understand the molecular basis of prion propagation, pathogenesis, and the barriers limiting intermammalian transmission. It is unclear if a large epidemic of variant CJD will occur in the years ahead.
journal_name
Annu Rev Neuroscijournal_title
Annual review of neuroscienceauthors
Collinge Jdoi
10.1146/annurev.neuro.24.1.519keywords:
subject
Has Abstractpub_date
2001-01-01 00:00:00pages
519-50eissn
0147-006Xissn
1545-4126pii
24/1/519journal_volume
24pub_type
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journal_title:Annual review of neuroscience
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pub_type: 杂志文章
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更新日期:2020-07-08 00:00:00
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更新日期:1979-01-01 00:00:00
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pub_type: 杂志文章,评审
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更新日期:1999-01-01 00:00:00
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更新日期:2008-01-01 00:00:00
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journal_title:Annual review of neuroscience
pub_type: 杂志文章,评审
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更新日期:2000-01-01 00:00:00
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journal_title:Annual review of neuroscience
pub_type: 杂志文章,评审
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更新日期:2000-01-01 00:00:00
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journal_title:Annual review of neuroscience
pub_type: 杂志文章,评审
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更新日期:1999-01-01 00:00:00
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更新日期:2003-01-01 00:00:00
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更新日期:1997-01-01 00:00:00
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journal_title:Annual review of neuroscience
pub_type: 杂志文章,评审
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更新日期:2005-01-01 00:00:00
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journal_title:Annual review of neuroscience
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更新日期:2018-07-08 00:00:00
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journal_title:Annual review of neuroscience
pub_type: 杂志文章,评审
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更新日期:2005-01-01 00:00:00
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更新日期:2011-01-01 00:00:00
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更新日期:2003-01-01 00:00:00
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更新日期:2019-07-08 00:00:00
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journal_title:Annual review of neuroscience
pub_type: 杂志文章,评审
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更新日期:2019-07-08 00:00:00
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journal_title:Annual review of neuroscience
pub_type: 杂志文章,评审
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更新日期:2001-01-01 00:00:00
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journal_title:Annual review of neuroscience
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更新日期:1985-01-01 00:00:00