Mad cow disease has had devastating consequences for bovine livestock populations, particularly in Europe, and for humans who have consumed contaminated beef products.
To date, there have been more than 200 recorded human fatalities worldwide due to mad cow disease. Creutzfeldt-Jacob disease, a low-incidence but always fatal disease, affects humans in all countries.
Prions consist mainly or entirely of an abnormal form of a normal cellular protein. They multiply by converting their normal counterparts into a likeness of themselves, which may aggregate to form deposits called amyloid. Accumulation of different kinds of amyloid plays a role in a wide range of neurodegenerative diseases, including Alzheimer's and Parkinson's diseases.
Currently, different varieties of prion strains are identified in mouse models according to incubation time, clinical symptoms, and localization of brain lesions.
In the new test developed by Lasmzas and Weissmann, a transgenic mouse line called Tga20 plays an important role, because it succumbs rapidly to prion disease.
"The prions primarily target the brainstem and the thalamus of this transgenic mouse, explaining why these animals have a shorter incubation time than their normal counterparts," Lasmzas said. "The prion aggregates also don't spread evenly to other brain regions, and their distribution is characteristic for different strains."
Importantly, the brain tissue can be subjected to the Cell Panel Assay, which unlike the current histological method, doesn't require time intensive examination of brain lesions and can be completed within two weeks, Lasmzas added. The test has been partially automated.
Development of the method provided the scientists with the opportunit
|Contact: Mika Ono|
Scripps Research Institute