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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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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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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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2014
Prions are generally regarded as the most reliable markers of so-called prion diseases. With the advent of the protein-only concept of the transmission of prion diseases, which is predicated on the idea that prions are the infectious agents causing prion disease, and with the 1997 Nobel Prize for Medicine awarded for the prion protein concept, prions ...
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Prions are generally regarded as the most reliable markers of so-called prion diseases. With the advent of the protein-only concept of the transmission of prion diseases, which is predicated on the idea that prions are the infectious agents causing prion disease, and with the 1997 Nobel Prize for Medicine awarded for the prion protein concept, prions ...
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1996
Abstract Human prion diseases include idiopathic, genetic, and acquired disorders. Heterogeneous clinicopathologic features make diagnosis challenging. Accurate diagnosis requires a combined clinical, neuropathologic, genetic, and biochemical approach. Neuropathologic assessment is performed following autopsy in most cases.
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Abstract Human prion diseases include idiopathic, genetic, and acquired disorders. Heterogeneous clinicopathologic features make diagnosis challenging. Accurate diagnosis requires a combined clinical, neuropathologic, genetic, and biochemical approach. Neuropathologic assessment is performed following autopsy in most cases.
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