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Title: Redox metals and oxidative abnormalities in human prion diseases
Authors: Petersen, Robert 
Siedlak, Sandra 
Lee, Hyoung-Gon 
Kim, Yong-Sun 
Nunomura, Akihiko 
Tagliavini, Fabrizio 
Ghetti, Bernardino 
Cras, Patrick 
Moreira, Paula 
Castellani, Rudy 
Guentchev, Marin 
Budka, Herbert 
Ironside, James 
Gambetti, Pierluigi 
Smith, Mark 
Perry, George 
Issue Date: 2005
Citation: Acta Neuropathologica. 110:3 (2005) 232-238
Abstract: Prion diseases are characterized by the accumulation of diffuse and aggregated plaques of protease-resistant prion protein (PrP) in the brains of affected individuals and animals. Whereas prion diseases in animals appear to be almost exclusively transmitted by infection, human prion diseases most often occur sporadically and, to a lesser extent, by inheritance or infection. In the sporadic cases (sporadic Creutzfeld-Jakob disease, sCJD), PrP-containing plaques are infrequent, whereas in transmitted (variant CJD) and inherited (Gerstmann-Straussler-Scheinker Syndrome) cases, plaques are a usual feature. In the current study, representative cases from each of the classes of human prion disease were analyzed for the presence of markers of oxidative damage that have been found in other neurodegenerative diseases. Interestingly, we found that the pattern of deposition of PrP, amyloid-ß, and redox active metals was distinct for the various prion diseases. Whereas 8-hydroxyguanosine has been shown to be increased in sCJD, and inducible NOS is increased in scrapie-infected mice, well-studied markers of oxidative damage that accumulate in the lesions of other neurodegenerative diseases (such as Alzheimer’s disease, progressive supranuclear palsy, and Parkinson’s disease), such as heme oxygenase-1 and lipid peroxidation, were not found around PrP deposits or in vulnerable neurons. These findings suggest an important distinction in prion-related oxidative stress, indicating that different neurodegenerative pathways are involved in different prion diseases.
DOI: 10.1007/s00401-005-1034-4
Rights: openAccess
Appears in Collections:FMUC Medicina - Artigos em Revistas Internacionais

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