Normal host prion protein necessary for scrapie-induced neurotoxicity

Article Abstract:

Scrapie and other prion diseases are characterized by the accumulation of a pathological and protease-resistant isoform of the normal host protein PrP(supra C) known as the prion protein PrP(supra Sc). A study is conducted to determine whether scrapie pathology is induced by a severe PrP(supra C) deficiency, by neurotoxicity of PrP(supra Sc) or by other mechanism. Neural tissue overexpressing PrP(supra C) is grafted into the brain of mice that are PrP-deficient and do not transmit infectivity. The results are discussed.

Analysis

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Unaltered susceptibility to BSE in transgenic mice expressing human prion protein

Article Abstract:

The human prion protein (PrP)-expressing transgenic mouse produces mouse PrP, human PrP (super Sc) and human PrP when exposed to bovine spongiform encephalopathy. Tg110 mice exhibit an incubation period of 484 +- 40 days to PDG170. The human PrP(super C) is less sensitive than mouse PrP(super C) in the bovine prion conversion to PrP(super Sc). Immunodotblots reveal the presence of human PrP(super Sc) in Creutzfeldt-Jakob disease-inoculated sick transgenic mice. Inoculation of HuPrP+/+ Prn-p(super 0/0) mice is discussed.

Genetic aspects, Creutzfeldt-Jakob disease, Genetically modified mice

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Monoclonal antibodies inhibit prion replication and delay the development of prion disease

Article Abstract:

An important advance in prion immunotherapeutics has been made by neuroscientists in England. Using a murine scrapie model, it was possible to significantly reduce the infective potential through use of monoclonal antibodies, resulting in animals that were still healthy 300 days after untreated test animals had died of the disease.

United Kingdom, Science & research, Care and treatment, Monoclonal antibodies, Immunotherapy

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