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The Lancet Neurology, 2005
Prion diseases are degenerative disorders of the nervous system caused by transmissible particles that contain a pathogenic isoform of the prion protein, a normal constituent of cell membranes. The most common human prion disease is Creutzfeldt-Jakob disease (CJD). Most cases are sporadic with unknown mode of transmission, 10-15% of cases are inherited,
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Prion diseases are degenerative disorders of the nervous system caused by transmissible particles that contain a pathogenic isoform of the prion protein, a normal constituent of cell membranes. The most common human prion disease is Creutzfeldt-Jakob disease (CJD). Most cases are sporadic with unknown mode of transmission, 10-15% of cases are inherited,
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Journal of Neurovirology, 2003
Prion diseases are incurable neurodegenerative conditions affecting both animals and humans. They may be sporadic, infectious, or inherited in origin. Human prion diseases include Creutzfeldt-Jakob desease (CJD), Gerstmann-Straussler-Scheinker disease, kuru, and fatal familial insomnia.
Edward, McKintosh +2 more
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Prion diseases are incurable neurodegenerative conditions affecting both animals and humans. They may be sporadic, infectious, or inherited in origin. Human prion diseases include Creutzfeldt-Jakob desease (CJD), Gerstmann-Straussler-Scheinker disease, kuru, and fatal familial insomnia.
Edward, McKintosh +2 more
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1996
List of Contents.- Human Prion Diseases and Neurodegeneration.- Fatal Familial Insomnia and Familial Creutzfeldt-Jakob Disease: A Tale of Two Diseases with the Same Genetic Mutation.- Human Prion Disease and Human Prion Protein Disease.- Prion Strains.- Structures of Prion Proteins and Conformational Models for Prion Diseases.- The Folding Intermediate
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List of Contents.- Human Prion Diseases and Neurodegeneration.- Fatal Familial Insomnia and Familial Creutzfeldt-Jakob Disease: A Tale of Two Diseases with the Same Genetic Mutation.- Human Prion Disease and Human Prion Protein Disease.- Prion Strains.- Structures of Prion Proteins and Conformational Models for Prion Diseases.- The Folding Intermediate
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The Prion Concept and Synthetic Prions
2017Transmissible spongiform encephalopathies or prion diseases are a group of fatal neurodegenerative diseases caused by unconventional infectious agents, known as prions (PrPSc). Prions derive from a conformational conversion of the normally folded prion protein (PrPC), which acquires pathological and infectious features.
Legname, Giuseppe, Moda, Fabio
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2012
For both mammalian and fungal prion proteins, conformational templating drives the phenomenon of protein-only infectivity. The conformational conversion of a protein to its transmissible prion state is associated with changes to host cellular physiology.
Gemma L, Staniforth, Mick F, Tuite
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For both mammalian and fungal prion proteins, conformational templating drives the phenomenon of protein-only infectivity. The conformational conversion of a protein to its transmissible prion state is associated with changes to host cellular physiology.
Gemma L, Staniforth, Mick F, Tuite
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Haemophilia, 1998
Summary. Although the nature of the infectious agent causing prion diseases is still debated, several of its molecular characteristics have been clarified in remarkable detail. The transmissibility of bovine spongiform encephalopathy to humans dramatically highlights the need for research focused at interference with prion replication and spread, and ...
C. A. Lee +6 more
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Summary. Although the nature of the infectious agent causing prion diseases is still debated, several of its molecular characteristics have been clarified in remarkable detail. The transmissibility of bovine spongiform encephalopathy to humans dramatically highlights the need for research focused at interference with prion replication and spread, and ...
C. A. Lee +6 more
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2018
Genetic prion diseases (gPrDs) are caused by autosomal-dominant mutations in the prion protein gene (PRNP). Although the first PRNP mutations identified, and most since, are PRNP missense, octapeptide repeat insertions, deletion and nonsense mutations have now also been shown to cause gPrD. Based on clinicopathologic features of familial disease, gPrDs
Leonel T, Takada +4 more
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Genetic prion diseases (gPrDs) are caused by autosomal-dominant mutations in the prion protein gene (PRNP). Although the first PRNP mutations identified, and most since, are PRNP missense, octapeptide repeat insertions, deletion and nonsense mutations have now also been shown to cause gPrD. Based on clinicopathologic features of familial disease, gPrDs
Leonel T, Takada +4 more
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Seminars in Neurology, 2019
AbstractPrion diseases are a phenotypically diverse set of disorders characterized by protease-resistant abnormally shaped proteins known as prions. There are three main groups of prion diseases, termed sporadic (Creutzfeldt–Jakob disease [CJD], sporadic fatal insomnia, and variably protease-sensitive prionopathy), genetic (genetic CJD, fatal familial ...
Kelly J, Baldwin, Cynthia M, Correll
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AbstractPrion diseases are a phenotypically diverse set of disorders characterized by protease-resistant abnormally shaped proteins known as prions. There are three main groups of prion diseases, termed sporadic (Creutzfeldt–Jakob disease [CJD], sporadic fatal insomnia, and variably protease-sensitive prionopathy), genetic (genetic CJD, fatal familial ...
Kelly J, Baldwin, Cynthia M, Correll
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Journal of Veterinary Medicine, Series B, 2006
SummaryThe prion theory postulates that prions are novel infectious agents that are composed largely, if not entirely, of abnormally folded host‐encoded prion proteins. However, the existence of different prion strains is enigma, if these novel infectious agents lack a genetic element, such as a nucleic acid.
M, Eiden +3 more
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SummaryThe prion theory postulates that prions are novel infectious agents that are composed largely, if not entirely, of abnormally folded host‐encoded prion proteins. However, the existence of different prion strains is enigma, if these novel infectious agents lack a genetic element, such as a nucleic acid.
M, Eiden +3 more
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Biochemical Society Symposia, 2004
Prion diseases, also referred to as transmissible spongiform encephalopathies, are characterized by the deposition of an abnormal isoform of the prion protein in the brain. However, this aggregated, fibrillar, amyloid protein, termed PrPSc, is an altered conformer of a normal brain glycoprotein, PrPc.
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Prion diseases, also referred to as transmissible spongiform encephalopathies, are characterized by the deposition of an abnormal isoform of the prion protein in the brain. However, this aggregated, fibrillar, amyloid protein, termed PrPSc, is an altered conformer of a normal brain glycoprotein, PrPc.
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