Back to Search Start Over

Characterization of Prion Proteins.

Authors :
Walker, John M.
Potter, Nicholas T.
Zou, Wenquan
Colucci, Monica
Gambetti, Pierluigi
Chen, Shu G.
Source :
Neurogenetics (9780896039902); 2003, p305-314, 10p
Publication Year :
2003

Abstract

Prion disease represents a group of transmissible neurodegenerative disorders that include scrapie in sheep and goats, bovine spongiform encephalopathy (BSE), and Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker syndrome, fatal familial insomnia, and kuru in humans. The disease is characterized clinically by ataxia, dementia, and myoclonus as well as pathologically by spongiosis, astrocytic gliosis, and neuronal loss (reviewed in refs. 1 and 2). CJD occurs mostly (about 85% of all cases) as sporadic cases (unknown etiology), with the rest of the cases being familial (owing to inheritance of mutations in the prion protein gene) or iatrogenic (due to accidental transmission during medical procedure). Recently, a new variant form of CJD (vCJD) has emerged in the United Kingdom that may originate from the BSE epidemic (3). [ABSTRACT FROM AUTHOR]

Details

Language :
English
ISBNs :
9780896039902
Database :
Supplemental Index
Journal :
Neurogenetics (9780896039902)
Publication Type :
Book
Accession number :
33258497
Full Text :
https://doi.org/10.1385/1-59259-330-5:305