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Transmissible spongiform encephalopathies (TSEs), also known as prion diseases, [1] are a group of progressive, incurable, and fatal conditions that are associated with prions and affect the brain and nervous system of many animals, including humans, cattle, and sheep.
All types of CJD are transmissible irrespective of how they occur in the person. [26] It is thought that humans can contract the variant form of the disease by eating food from animals infected with bovine spongiform encephalopathy (BSE), the bovine form of TSE also known as mad cow disease. However, it can also cause sCJD in some cases. [27] [28]
A prion / ˈ p r iː ɒ n / ⓘ is a misfolded protein that induces misfolding in normal variants of the same protein, leading to cellular death.Prions are responsible for prion diseases, known as transmissible spongiform encephalopathy (TSEs), which are fatal and transmissible neurodegenerative diseases affecting both humans and animals.
Kuru, a transmissible spongiform encephalopathy, is a disease of the nervous system that causes physiological and neurological effects which ultimately lead to death. It is characterized by progressive cerebellar ataxia, or loss of coordination and control over muscle movements. [11] [12]
Variant Creutzfeldt–Jakob disease (vCJD), formerly known as New variant Creutzfeldt–Jakob disease (nvCJD) and referred to colloquially as "mad cow disease" or "human mad cow disease" to distinguish it from its BSE counterpart, is a fatal type of brain disease within the transmissible spongiform encephalopathy family. [7]
It is, however, classified with the transmissible spongiform encephalopathies (TSE) due to the causative role played by PRNP, the human prion protein. [2] GSS was first reported by the Austrian physicians Josef Gerstmann, Ernst Sträussler and Ilya Scheinker in 1936. [3] [4] Familial cases are associated with autosomal-dominant inheritance. [5]
Pages in category "Transmissible spongiform encephalopathies" The following 18 pages are in this category, out of 18 total. This list may not reflect recent changes. ...
A form of transmissible spongiform encephalopathy specific to humans, known as Creutzfeldt-Jakob disease (CJD), is a degeneration of the central nervous system characterized by the accumulation of a prion. The incubation period lasts years, even decades, before balance and sensitivity disorders appear, followed by dementia. The outcome is ...