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Towards a treatment for genetic prion disease: trials and biomarkers.
Lancet Neurology, 2020Prion disease is a rare, fatal, and exceptionally rapid neurodegenerative disease. Although incurable, prion disease follows a clear pathogenic mechanism, in which a single gene gives rise to a single prion protein (PrP) capable of converting into the ...
S. M. Vallabh +3 more
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Current Biology, 1992
There have been remarkably rapid advances in the understanding of prion diseases over the past year. The controversial notion that the transmissible agent may be an abnormal isoform of a host-encoded protein, the prion protein, is now gaining wide acceptance.
J, Collinge, M S, Palmer
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There have been remarkably rapid advances in the understanding of prion diseases over the past year. The controversial notion that the transmissible agent may be an abnormal isoform of a host-encoded protein, the prion protein, is now gaining wide acceptance.
J, Collinge, M S, Palmer
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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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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
openaire +3 more sources
Neurologic Clinics, 2018
Prions diseases are uniformly fatal neurodegenerative diseases that occur in sporadic, genetic, and acquired forms. Acquired prion diseases, caused by infectious transmission, are least common. Most prion diseases are not infectious, but occur spontaneously through misfolding of normal prion proteins or genetic mutations in the prion protein gene ...
Boon Lead, Tee +2 more
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Prions diseases are uniformly fatal neurodegenerative diseases that occur in sporadic, genetic, and acquired forms. Acquired prion diseases, caused by infectious transmission, are least common. Most prion diseases are not infectious, but occur spontaneously through misfolding of normal prion proteins or genetic mutations in the prion protein gene ...
Boon Lead, Tee +2 more
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Prion disease incidence in the United States: 2003–2015
Neurology, 2019Objective To report the incidence of prion disease in the United States. Methods Prion disease decedents were retrospectively identified from the US national multiple cause-of-death data for 2003–2015 and matched with decedents in the National Prion ...
Ryan A. Maddox +6 more
semanticscholar +1 more source
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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International Ophthalmology Clinics, 2007
Prion diseases are a unique group of neurologic diseases caused by an abnormal protein conformation. Prion diseases encompass genetic, sporadic, iatrogenic, and acquired conditions in humans and other mammals. Although they are relatively rare, they produce a diverse array of symptoms, uniformly are fatal, and provide important information about ...
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Prion diseases are a unique group of neurologic diseases caused by an abnormal protein conformation. Prion diseases encompass genetic, sporadic, iatrogenic, and acquired conditions in humans and other mammals. Although they are relatively rare, they produce a diverse array of symptoms, uniformly are fatal, and provide important information about ...
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Prion Diseases and Emerging Prion Diseases
Current Medicinal Chemistry, 2008Transmissible spongiform encephalopathies (TSEs), also called prion diseases, are fatal neurodegenerative disorders. An abnormal isoform of the prion protein (PrP(Sc)) generated by post-translational modification of the cellular prion protein (PrP(C)) is believed to be the main component of this infectious agent.
Takashi, Yokoyama, Shirou, Mohri
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Journal of Geriatric Psychiatry and Neurology, 2010
The prion diseases are a family of rare neurodegenerative disorders that result from the accumulation of a misfolded isoform of the prion protein (PrP), a normal constituent of the neuronal membrane. Five subtypes constitute the known human prion diseases; kuru, Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker syndrome (GSS), fatal ...
Khalilah, Brown, James A, Mastrianni
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The prion diseases are a family of rare neurodegenerative disorders that result from the accumulation of a misfolded isoform of the prion protein (PrP), a normal constituent of the neuronal membrane. Five subtypes constitute the known human prion diseases; kuru, Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker syndrome (GSS), fatal ...
Khalilah, Brown, James A, Mastrianni
openaire +2 more sources