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Molecular Mechanisms Encoding Strains of Prions and Prion-Like Misfolded Proteins
Yeast, fungal, and mammalian prions determine heritable as well as infectious traits (Shorter J, Lindquist S. Nat Rev Genet, 6:435–450, 2005; Wickner...
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Prion strains viewed through the lens of cryo-EM
Mammalian prions are lethal transmissible pathogens that cause fatal neurodegenerative diseases in humans and animals. They consist of fibrils of...
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Prion strains: shining new light on old concepts
Prion diseases are a group of inevitably fatal neurodegenerative disorders affecting numerous mammalian species, including humans. The existence of...
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Prion Strain Interference
Prions are transmissible agents comprised of a misfolded protein PrPSc that is post-translationally derived from the normal isoform PrPC. Prion...
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PrP Prion Structures
The biophysical properties of authentic infectious prion protein (PrP)-based mammalian prions have long impeded determination of their detailed...
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Cell biology of prion strains in vivo and in vitro
The properties of infectious prions and the pathology of the diseases they cause are dependent upon the unique conformation of each prion strain. How...
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Human Prion Disease Surveillance
Human prion diseases are characterized by rapid fatal neurodegeneration caused by pathologic prion proteins that are transmissible under specific...
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Combination of Styrylbenzoazole Compound and Hydroxypropyl Methylcellulose Enhances Therapeutic Effect in Prion-Infected Mice
Prion diseases are fatal transmissible neurodegenerative disorders. Tremendous efforts have been made for prion diseases; however, no effective...
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Species Barriers in Prion Disease
Species barriers in prion diseases are defined by the difficulty that prions from one species have in triggering prion infection in a new species....
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Prion Conversion and Deformed Templating
The transmissible agent of prion disease consists of a prion protein in its abnormal, β-sheet-rich state (PrPSc), which replicates itself according...
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Prion Protein Conversion and Lipids
The conversion of α-helical rich normal prion protein to a β-sheeted pathogenic isoform is central to prion disease. Decades of studies provided...
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Human Sporadic Prion Diseases
Sporadic or idiopathic prion diseases account for over 90% of all human prion diseases, and sporadic Creutzfeldt–Jakob disease (sCJD) is by far the...
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Cofactor Involvement in Prion Propagation
Pure amyloid proteins are responsible for the transmissible properties of yeast prions (Tanaka et al., Nature 428(6980):323–328, 2004; Cell...
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Accumulation of Prion Triggers the Enhanced Glycolysis via Activation of AMKP Pathway in Prion-Infected Rodent and Cell Models
Mitochondrial dysfunction is one of the hallmarks in the pathophysiology of prion disease and other neurodegenerative diseases. Various metabolic...
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Drosophila Models of Prion Diseases
Prion diseases encompass a heterogeneous group of fatal brain disorders associated with the accumulation of misfolded isoforms of the prion protein...
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Overview on Treatment of Prion Diseases and Decontamination of Prions
Currently, there are no prophylactic or disease-modifying therapies for prion diseases with proven, significant efficacy. The discovery of treatments...
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Yeast Prions Are Folded, In-Register Parallel Amyloids Subject to Multiple Anti-prion Systems
Most yeast prions are self-propagating amyloids of normally non-amyloid proteins. The prion domains of Ure2p, Sup35p, and Rnq1p each form highly...
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Prion strains associated with iatrogenic CJD in French and UK human growth hormone recipients
Treatment with human pituitary-derived growth hormone (hGH) was responsible for a significant proportion of iatrogenic Creutzfeldt–Jakob disease...
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New developments in prion disease research using genetically modified mouse models
While much of what we know about the general principles of protein-based information transfer derives from studies of experimentally adapted rodent...
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Comparing Prion Proteins Across Species: Is Zebrafish a Useful Model?
Despite the considerable body of research dedicated to the field of neurodegeneration, the gap in knowledge on the prion protein and its intricate...
