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TSEs in non-human mammals include scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle – popularly known as "mad cow disease" – and chronic wasting disease (CWD) in deer and elk. The variant form of Creutzfeldt–Jakob disease in humans is caused by exposure to bovine spongiform encephalopathy prions. [4] [5] [6]
Creutzfeldt–Jakob disease (CJD), also known as subacute spongiform encephalopathy or neurocognitive disorder due to prion disease, is a fatal neurodegenerative disease. [ 4 ] [ 1 ] Early symptoms include memory problems, behavioral changes, poor coordination, and visual disturbances. [ 4 ]
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]
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.
Human growth hormone derived from cadavers was linked to Alzheimer's disease in five patients who received injections decades ago, ... CJD is a cousin of bovine spongiform encephalopathy, commonly ...
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]
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 ...
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]