Metabolic aspects of prion diseases: an overview.
Prion diseases are fatal neurodegenerative disorders that affect humans and other mammals. The hallmark of these diseases is the conformational change of the cellular prion protein (PrP(C)) to the misfolded protein capable of propagation and associated with neurodegenration, named prion (PrP(Sc)). In a strict sense, prion diseases are a consequence of aberrations in the metabolism of the cellular prion protein (PrP(C)). This brief review addresses current understanding of metabolic disturbances in prion disorders at the cellular, organ and organism level, selectively pointing out some relevant diagnostic and treatment options.
Vranac
Tanja
T
Blood Transfusion Centre of Slovenia, Slajmerjeva 6, 1000 Ljubljana, Slovenia.
Bresjanac
Mara
M
D000818:Animals; D006801:Humans; D018621:PrPC Proteins / Q000378:metabolism*; D018620:PrPSc Proteins / Q000378:metabolism*; D017096:Prion Diseases / Q000175:diagnosis / Q000503:physiopathology* / Q000628:therapy; D017510:Protein Folding
D018621:PrPC Proteins; D018620:PrPSc Proteins