Author/Editor     Vranac, Tanja; Bresjanac, Mara
Title     Metabolic aspects of prion diseases: an overview
Type     članek
Source     Curr Drug Targets
Vol. and No.     Letnik 11, št. 10
Publication year     2010
Volume     str. 1207-17
Language     eng
Abstract     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.
Descriptors     PRION DISEASES
PRIONS
PRPC PROTEINS
PRPSC PROTEINS
NERVE DEGENERATION
TRANSLATION, GENETIC
BRAIN
NUCLEAR MAGNETIC RESONANCE
TOMOGRAPHY, EMISSION-COMPUTED