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Transmissible spongiform encephalopathies

The Lancet, 2004
Nosologically, transmissible spongiform encephalopathies (TSE or prion diseases) should be grouped with other neurodegenerative disorders such as Alzheimer's and Parkinson's diseases, which are all caused by toxic gain of function of an aberrant form of a constitutively expressed protein.
Steven J, Collins   +2 more
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Transmissible Spongiform Encephalopathies

Biochemical and Biophysical Research Communications, 1998
Scrapie, bovine spongiform encephalopathy (BSE), and the Creutzfeldt-Jakob disease (CJD) belong to a group of lethal neurodegenerative disorders in mammals. Prion diseases or transmissible spongiform encephalopathies (TSEs) are characterized by the accumulation of an abnormal isoform (PrPSc) of the host-encoded cellular prion protein (PrPC) in the ...
S, Liemann, R, Glockshuber
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THE TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES

Annual Review of Medicine, 1995
▪ Abstract  The human transmissible spongiform encephalopathies (TSEs), or prion diseases, are a group of rapidly progressive disorders characterized by a spectrum of clinical abnormalities that include cognitive impairment, ataxia, myoclonus, and visual, pyramidal, and extrapyramidal signs. They share a spongiform (vacuolar) degeneration and variable
L G, Goldfarb, P, Brown
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Transmissible Spongiform Encephalopathies

2007
Protein folding and misfolding, relevance to disease and function / Massimi Stefani -- Alzheimer's disease / Charlotte E. Teunissen and Tischa M. van der Cammen -- Improving Cholinergic Transmission -- Cholinergic transmission and acetylcholine release enhancers / Pierre Francotte, Pascal de Tullio and Bernard Pirotte -- AChE and its inhibition / Jure ...
Geschwind, M. D., Legname, G.
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Transmissible Spongiform Encephalopathies

Journal of Food Science, 2005
The Institute of Food Technologists has issued this Scientific Status Summary to update our knowledge of transmissible spongiform encephalopathies and provide an authoritative perspective on the surrounding regulatory and trade landscape.
Will Hueston, Cory M. Bryant
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Transmissible spongiform encephalopathies

2016
Prion disease is a unique category of illness, affecting both animals and humans, in which the underlying pathogenesis is related to a conformational change of a normal, self-protein called PrPC (C for cellular) to a pathological and infectious conformer known as PrPSc (Sc for scrapie) (Prusiner, Annu Rev Genet 47:601–623, 2013; Aguzzi and Falsig, Nat ...
Thomas Wisniewski, Fernando Goñi
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Transmissible spongiform encephalopathy

Reviews in Medical Microbiology, 1998
Transmissible spongiform encephalopaties (TSE) are a group of rare fatal diseases of humans and animals. Prions, small infectious proteinaceous particles, are their supposed cause. Prusiner's theory (Nobel price 1997) proves that pathogenetically active prions are conformated physiological prions but problems of pathogenesis of TSE are still open. Most
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Transmissible Spongiform Encephalopathies

2015
Prion diseases, also termed transmissible spongiform encephalopathies (TSEs), are inevitably fatal neurodegenerative conditions which affect humans and a wide variety of animals. The most common form of prion disease in humans is sporadic Creutzfeldt-Jakob disease (sCJD).
Markus Glatzel, Adriano Aguzzi
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