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Prion Disease

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

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

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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Copper and prion diseases

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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Prion Protein Disease and Neuropathology of Prion Disease

Neuroimaging Clinics of North America, 2008
Human 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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The stress of prion disease

Brain Research, 2016
Prion diseases are fatal neurodegenerative disorders that include scrapie of sheep, bovine spongiform encephalopathy of cattle, chronic wasting disease of cervids, and Creutzfeldt-Jakob disease (CJD) of humans. The etiology for prion diseases can be infectious, sporadic, or hereditary. However, the common denominator for all types is the formation of a
Charles E, Mays, Claudio, Soto
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Prion diseases

European Neuropsychopharmacology, 2022
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Prions and Prion Diseases

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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Human Prion Diseases

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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Prions and prion diseases.

Acta medica Croatica : casopis Hravatske akademije medicinskih znanosti, 1997
Currently known transmissive spongiform encephalopathies in humans and animals are presented. Caused by prions, they are known as "prion disease". The results of extensive investigations of prions are described, as well as the theories about their nature.
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