Prion-induced neuronal damage—the mechanisms of neuronal destruction in the subacute spongiform encephalopathies
A Giese, HA Kretzschmar - The Mechanisms of Neuronal Damage in Virus …, 2001 - Springer
A Giese, HA Kretzschmar
The Mechanisms of Neuronal Damage in Virus Infections of the Nervous System, 2001•SpringerThe transmissible spongiform encephalopathies, or prion diseases, constitute a group of
transmissible, rapidly progressive, invariably fatal neurodegenerative diseases that can
manifest as acquired, hereditary or idiopathic (“sporadic”) disease. They include Creutzfeldt-
Jakob disease in humans as well as scrapie and bovine spongiform encephalopathy (BSE)
in animals, and are characterized by a long incubation period which may last up to decades
after experimental or accidental transmission. The classic pathological features of prion …
transmissible, rapidly progressive, invariably fatal neurodegenerative diseases that can
manifest as acquired, hereditary or idiopathic (“sporadic”) disease. They include Creutzfeldt-
Jakob disease in humans as well as scrapie and bovine spongiform encephalopathy (BSE)
in animals, and are characterized by a long incubation period which may last up to decades
after experimental or accidental transmission. The classic pathological features of prion …
Abstract
The transmissible spongiform encephalopathies, or prion diseases, constitute a group of transmissible, rapidly progressive, invariably fatal neurodegenerative diseases that can manifest as acquired, hereditary or idiopathic (“sporadic”) disease. They include Creutzfeldt-Jakob disease in humans as well as scrapie and bovine spongiform encephalopathy (BSE) in animals, and are characterized by a long incubation period which may last up to decades after experimental or accidental transmission. The classic pathological features of prion diseases include spongiform change, gliosis and neuronal loss. In contrast to what is typically seen in infectious diseases caused by viruses, they lack a significant inflammatory response (Prusiner 1993, 1998).
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