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
Current Drug Targets
Title: Metabolic Aspects of Prion Diseases: An Overview
Volume: 11 Issue: 10
Author(s): Tanja Vranac and Mara Bresjanac
Affiliation:
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
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.
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Cite this article as:
Vranac Tanja and Bresjanac Mara, Metabolic Aspects of Prion Diseases: An Overview, Current Drug Targets 2010; 11 (10) . https://dx.doi.org/10.2174/1389450111007011207
DOI https://dx.doi.org/10.2174/1389450111007011207 |
Print ISSN 1389-4501 |
Publisher Name Bentham Science Publisher |
Online ISSN 1873-5592 |
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