Metabolic Aspects of Prion Diseases: An Overview

Tanja      Vranac; Mara      Bresjanac

doi:10.2174/1389450111007011207

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 (PrPC) to the misfolded protein capable of propagation and associated with neurodegeneration, named prion (PrPSc). In a strict sense, prion diseases are a consequence of aberrations in the metabolism of the cellular prion protein (PrPC). 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.

Keywords: Conformational diseases, metabolism, neurodegeneration, PrPC, PrPSc, transmissible spongiform encephalopathies, Prion diseases, Prion, Isoform, Glycoprotein, Misfolding proteins, Amyloid-β, PRNP gene, Translocation, Cytoplasm, Homeostasis, Olfactory discrimination, Embryogenesis, Truncations, Parkinson disease, Alzhemier disease, Tauopathies, Proteinopathies, Follicular dendritic cells, Lymphotoxin, Endocytosis, Monoclonal antibodies, Motif-grated antibodies, Epitope metabolism, ER stress, Oxidatie stress, Creutzfeldt-Jakob disease, Dementia, Myoclonus, Gerstmann-Straussler-Scheinker disease, Ataxia, Magnetic resonance spectroscopy, Pulvinar sign, Myoinositol, Gliosis, PET, Quinacrine, Swansonine, Darwinian evoluition, Anti-idiotypic antibodies