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Mouse Models of Prion Protein Related Diseases
2011Prion protein (PrP) related diseases are a heterogeneous group of fatal neurodegenerative conditions featuring aberrancies in the metabolism of the cellular prion protein (PrPC) that lead to the formation of neurotoxic or propagative conformers. Commonly referred to as transmissible spongiform encephalopaties or prion disorders, these diseases can be ...
María Gasset, Adriano Aguzzi
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Prion-protein-related diseases of animals and man
2011Scrapie, bovine spongiform encephalopathy (BSE), Creutzfeldt–Jakob disease (CJD), and related diseases of mink (transmissible mink encephalopathy), mule deer and elk (chronic wasting disease) are the founder members of a group of diseases called the transmissible degenerative (or spongiform) encephalopathies (TSE).
James Hope, Mark P. Dagleish
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Amyloid in alzheimer's disease and prion-related encephalopathies: Studies with synthetic peptides
Progress in Neurobiology, 1996Deposition of amyloid-beta protein (beta A) in brain parenchyma and vessel walls is a major pathological feature of Alzheimer's disease (AD). In prion-related encephalopathies (PRE), too, an altered form of prion protein (PrPsc) forms amyloid fibrils and accumulates in the brain.
G, Forloni +3 more
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Journal of Molecular Biology, 2010
The prion protein can exist both in a normal cellular isoform and in a pathogenic conformational isoform. The latter is responsible for the development of different neurodegenerative diseases, for example Creutzfeldt-Jakob disease or fatal familial insomnia.
Christian, Gerum +2 more
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The prion protein can exist both in a normal cellular isoform and in a pathogenic conformational isoform. The latter is responsible for the development of different neurodegenerative diseases, for example Creutzfeldt-Jakob disease or fatal familial insomnia.
Christian, Gerum +2 more
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Rejuvenation Research, 2008
Prion diseases, Alzheimer's disease, and Parkinson's disease are age-related neurodegenerative diseases that are characterized by the formation of protein aggregates during the progress of the disease. Although it is still not known whether these aggregates are causative for, or symptoms of, the disease.
Eva, Birkmann +5 more
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Prion diseases, Alzheimer's disease, and Parkinson's disease are age-related neurodegenerative diseases that are characterized by the formation of protein aggregates during the progress of the disease. Although it is still not known whether these aggregates are causative for, or symptoms of, the disease.
Eva, Birkmann +5 more
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Prion Disorders: Creutzfeldt-Jakob Disease and Related Disorders
2021Abstract Prion disorders, or transmissible spongiform encephalopathies (TSEs), are universally fatal human and animal diseases that cause rapid degeneration of brain neurons by way of a conformational change in the prion protein that autocatalyzes further conformational change and selective neuronal toxicity. TSEs may occur sporadically,
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Prion's Progress: Patterns and Rates of Molecular Evolution in Relation to Spongiform Disease
Journal of Molecular Evolution, 1998Modification of the cellular prion protein has been correlated with the acquisition of several neurodegenerative diseases, including kuru, scrapie, bovine spongiform encephalopathy (BSE), and Creutzfeldt-Jakob disease (CJD). Sequence conservation and amino acid identity are known to influence the efficacy of interspecific transmission.
D C, Krakauer, P M, Zanotto, M, Pagel
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Epitope scanning indicates structural differences in brain-derived monomeric and aggregated mutant prion proteins related to genetic prion diseases [PDF]
Biochemical Journal, 2013 Genetic Creutzfeldt–Jakob disease, Gerstmann–Sträussler–Scheinker syndrome, fatal familial insomnia and prion protein cerebral amyloid angiopathy are clinically and neuropathologically distinct neurodegenerative diseases linked to mutations in the PRNP gene encoding the cellular prion protein (PrPC). How sequence variants of PRNP encode the information
Tapella L. +5 more
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Biochemistry, 1999
Transmissible spongiform encephalopathies (TSEs) are caused by a unique infectious agent which appears to be identical with PrPSc, an oligomeric, misfolded isoform of the cellular prion protein, PrPC. All inherited forms of human TSEs, i.e., familial Creutzfeldt-Jakob disease, Gerstmann-Sträussler-Scheinker syndrome, and fatal familial insomnia ...
S, Liemann, R, Glockshuber
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Transmissible spongiform encephalopathies (TSEs) are caused by a unique infectious agent which appears to be identical with PrPSc, an oligomeric, misfolded isoform of the cellular prion protein, PrPC. All inherited forms of human TSEs, i.e., familial Creutzfeldt-Jakob disease, Gerstmann-Sträussler-Scheinker syndrome, and fatal familial insomnia ...
S, Liemann, R, Glockshuber
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ChemInform Abstract: Molecular and Chemical Basis of Prion‐Related Diseases
ChemInform, 1998AbstractChemInform is a weekly Abstracting Service, delivering concise information at a glance that was extracted from about 100 leading journals. To access a ChemInform Abstract of an article which was published elsewhere, please select a “Full Text” option. The original article is trackable via the “References” option.
S. B. L. NG, A. J. DOIG
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