Journal
JOURNAL OF NEUROCHEMISTRY
Volume 97, Issue 6, Pages 1726-1739Publisher
WILEY
DOI: 10.1111/j.1471-4159.2006.03909.x
Keywords
immunology; inflammation; prions; transmissible spongiform encephalopathy
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Neuronal vacuolation (spongiosis), neuronal death, and pronounced glial reactions are the hallmarks of transmissible spongiform encephalopathies (TSEs), or prion diseases. A wealth of physical, biochemical, and immunological evidence indicates that the TSE agent, termed prion, does not contain agent-specific nucleic acid encoding its own constituents, as is the case for all other infectious pathogens. Also, no adaptive immune responses are elicited upon infection. A defining feature of TSEs is the deposition, mainly in the brain and lymphoreticular tissues, of an aggregated and structurally abnormal protein, designated PrPSc or PrP-res, which represents a conformational isomer of the ubiquitous surface protein PrPC. Biochemical and genetic evidence link PrP and its gene to the disease. Although TSEs are by definition transmissible, a growing number of Prnp-associated non-infectious neurodegenerative proteinopathies are now being recognized.
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