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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 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
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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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Neurologic 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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Seminars in Neurology, 2000
The prion diseases constitute an unusual group of neurodegenerative disorders. Although they are similar in many ways to other more common diseases, such as Alzheimer disease and amyotrophic lateral sclerosis, they are set apart on the basis of their transmissible nature.
J A, Mastrianni, R P, Roos
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The prion diseases constitute an unusual group of neurodegenerative disorders. Although they are similar in many ways to other more common diseases, such as Alzheimer disease and amyotrophic lateral sclerosis, they are set apart on the basis of their transmissible nature.
J A, Mastrianni, R P, Roos
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Clinics in Laboratory Medicine, 2010
Prion diseases are disorders affecting the central nervous system caused by alterations in the conformation of the cellular prion protein. They can be sporadic, hereditary, or acquired and usually present with myoclonus and rapidly progressive dementia in human patients.
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Prion diseases are disorders affecting the central nervous system caused by alterations in the conformation of the cellular prion protein. They can be sporadic, hereditary, or acquired and usually present with myoclonus and rapidly progressive dementia in human patients.
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2017
Prion diseases are a group of invariably fatal and transmissible neurodegenerative disorders that are associated with the misfolding of the normal cellular prion protein, with the misfolded conformers constituting an infectious unit referred to as a "prion".
Benjamin C, Whitechurch +3 more
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Prion diseases are a group of invariably fatal and transmissible neurodegenerative disorders that are associated with the misfolding of the normal cellular prion protein, with the misfolded conformers constituting an infectious unit referred to as a "prion".
Benjamin C, Whitechurch +3 more
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Biochemical Society Transactions, 2001
Transmissible spongiform encephalopathies are diseases of animals and humans that are also termed prion diseases. These diseases are linked together because a normal brain glycoprotein termed the prion protein is converted to a readily detectable protease-resistant isoform.
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Transmissible spongiform encephalopathies are diseases of animals and humans that are also termed prion diseases. These diseases are linked together because a normal brain glycoprotein termed the prion protein is converted to a readily detectable protease-resistant isoform.
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Prion Protein Disease and Neuropathology of Prion Disease
Neuroimaging Clinics of North America, 2008Human prion diseases, in common with other neurodegenerative diseases, may be sporadic or inherited and are characterized by the accumulation of cellular proteins accompanied by neuronal death and synaptic loss. Prion diseases are, however, unique in being transmissible.
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Pathogenesis of prion diseases
Acta Neuropathologica, 2005Prion diseases are rare neurological disorders that may be of genetic or infectious origin, but most frequently occur sporadically in humans. Their outcome is invariably fatal. As the responsible pathogen, prions have been implicated. Prions are considered to be infectious particles that represent mainly, if not solely, an abnormal, protease-resistant ...
Ursula, Unterberger +2 more
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