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A prion is a small infectious particle, which resist inactivation by procedures that modify nucleic acids. Transmissible spongiform encephalopathies (TSEs also known as prion diseases) are a group of progressive conditions that affects the brain and nervous system of humans and animals and are transmitted by prions.
Obi, R K, Nwanebu, F C
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Synthetic prions generated in vitro are similar to a newly identified subpopulation of PrPSc from sporadic Creutzfeldt-Jakob disease [PDF]
In recent studies, the amyloid form of recombinant prion protein (PrP) encompassing residues 89-230 (rPrP 89-230) produced in vitro induced transmissible prion disease in mice.
Bocharova, O V +9 more
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Prions and Prion Diseases [PDF]
Prion diseases (transmissible encephalopathies) are progressive neurodegenerative diseases of animals and humans caused by a unique agent, the prion. These diseases have recently been in the news because of the emergence of bovine spongiform encephalopathy (BSE; mad cow disease) and variant Creutzfeldt-Jakob disease (vCJD) in the United Kingdom.
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Endogenous Viral Etiology of Prion Diseases [PDF]
Transmissible spongiform encephalopathies (TSEs), or prion diseases, are a group of incurable neurodegenerative disorders, including Kuru and Creutzfeldt-Jakob disease in humans, “mad cow” disease in cattle, and scrapie in sheep. This paper
Claudiu I. Bandea
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Incidence and spectrum of sporadic Creutzfeldt-Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification [PDF]
Six subtypes of sporadic Creutzfeldt-Jakob disease with distinctive clinico-pathological features have been identified largely based on two types of the abnormal prion protein, PrPSc, and the methionine (M)/valine (V) polymorphic codon 129 of the prion ...
Maurizio Pocchiari +51 more
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The crystal structure of the globular domain of sheep prion protein [PDF]
The prion protein PrP is a naturally occurring polypeptide that becomes transformed from a normal conformation to that of an aggregated form, characteristic of pathological states in fatal transmissible spongiform conditions such as Creutzfeld–Jacob ...
Vasisht N +28 more
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Background Prion diseases are zoonotic diseases with a broad infection spectrum among mammalian hosts and are caused by the misfolded prion protein (PrPSc) derived from the normal prion protein (PrPC), which encodes the prion protein gene (PRNP ...
Yong-Chan Kim +2 more
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Human Prion Disease and Human Prion Protein Disease [PDF]
Creutzfeldt-Jakob disease (CJD), kuru, and Gerstmann-Straussler syndrome (GSS) show clinical and pathological characteristics similar to those of scrapie, a transmissible neurodegenerative disease of sheep and goats. These diseases are caused by slow infectious agents designated as prions (PRUSINER 1982). The major component of prions is prion protein (
T, Kitamoto, J, Tateishi
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Prion and prion-like diseases in animals
Transmissible spongiform encephalopaties (TSEs) are fatal neurodegenerative diseases characterized by the aggregation and accumulation of the misfolded prion protein in the brain. Other proteins such as β-amyloid, tau or Serum Amyloid-A (SAA) seem to share with prions some aspects of their pathogenic mechanism; causing a variety of so called prion-like
Aguilar-Calvo, Patricia +4 more
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Characterization of variably protease-sensitive prionopathy by capillary electrophoresis
Variably Protease Sensitive Prionopathy (VPSPr) is a rare human prion disease that, like Creutzfeldt-Jakob disease (CJD), results in the deposition of abnormally folded prion protein aggregates in the brain and is ultimately fatal.
Jennifer Myskiw +7 more
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