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Continuum, 2015 This article presents an update on the clinical aspects of human prion disease, including the wide spectrum of their presentations.Prion diseases, a group of disorders caused by abnormally shaped proteins called prions, occur in sporadic (Jakob-Creutzfeldt disease), genetic (genetic Jakob-Creutzfeldt disease, Gerstmann-Sträussler-Scheinker syndrome ...
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Seminars in Neurology, 2012 Prion diseases are a group of diseases caused by abnormally conformed infectious proteins, called prions. They can be sporadic (Jakob-Creutzfeldt disease [JCD]), genetic (genetic JCD, Gerstmann-Sträussler-Scheinker, and familial fatal insomnia), or acquired (kuru, variant JCD, and iatrogenic JCD).
Takada, Leonel, Geschwind, Michael
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Rinsho Shinkeigaku, 2010 Human prion diseases are classified into 3 categories according to etiologies: idiopathic of unknown cause, acquired of infectious origin, and genetic by PRNP mutation. The surveillance committee have analyzed 2,494 cases and identified 1,402 as prion diseases.
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European Journal of Human Genetics, 2006 Prion diseases have stimulated intense scientific scrutiny since it was proposed that the infectious agent was devoid of nucleic acid. Despite this finding, genetics has played a key role in understanding the pathobiology and clinical aspects of prion disease through the effects of a series of polymorphisms and mutations in the prion protein gene (PRNP)
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Human Prion Disease and Human Prion Protein Disease [PDF]
, 1996 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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Journal of Neurology, Neurosurgery & Psychiatry, 2004 R S G, Knight, R G, Will
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Current Opinion in Genetics & Development, 2011 Prion diseases or transmissible spongiform encephalopathies (TSEs) are neurodegenerative disorders of humans and animals for which there are no effective treatments or cure. They include Creutzfeldt-Jakob disease (CJD) in humans and sheep scrapie, bovine spongiform encephalopathy (BSE) and chronic wasting disease (CWD) in cervids.
Lloyd, Sarah E +2 more
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Scientific American, 1995 The human prion diseases are fatal neurodegenerative maladies that may present as sporadic, genetic, or infectious illnesses. The sporadic form is called Creutzfeldt-Jakob disease (CJD) while the inherited disorders are called familial (f) CJD, Gerstmann-Straussler-Scheinker (GSS) disease and fatal familial insomnia (FFI).
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Prions and Prion Diseases [PDF]
Laboratory Medicine, 1999 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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