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How do prion diseases spread between different types of animals?

New research may explain how prion diseases like BSE spread between various types of animalsA discovery has been made by medical researchers at the University of Alberta which may explain how prion diseases such as Feline Spongiform Encephalopathy and Bovine Spongiform Encephalopathy (BSE), adapt to spread between various types of animals.

Prion diseases, collectively known as transmissible spongiform encephalopathies (TSEs), are a family of rare progressive neurodegenerative disorders which can affect both humans and animals. Prions are infectious agents made up of proteins in a misfolded format, causing neurodegeneration. Symptoms of neurodegenerative disorders include dementia, ataxia and convulsions. Although a vaccine has been developed in mice, neurodegenerative diseases are untreatable and fatal in other animals and humans.

Neurologist Dr. Valeria Sim and her research team have discovered a previously undiscovered microscopic change in the prions framework which appears to allow the disease the ability to adapt. This flexibility in adaption allows prions to mimic and recreate new strains with which they may come into contact with.  Although prion diseases don’t always move from one animal to another successfully, when they do, it is called ‘adaption’. Dr. Sim aims to figure out what triggers this process to happen – “we want to determine why one prion disease might be able to spread from one type of animal to another, and why another strain of the disease can’t”. One of the realisations of this research is that if strains of prion disease are passed through a number different hosts, the disease can adapt and increase the number of susceptible hosts.

And this is a major concern. Since 1986, BSE has killed over 179,000 cattle, with another 4.4 million destroyed as a precaution at a cost of £5 billion from the taxpayer. If prions continue to adapt, more animals and humans are likely to be infected with new variants of diseases. Particular attention needs to be paid to chronic wasting disease, a TSE of deer and elk. This disease is worrying as the prions excreted from deer and elk are deposited into soil and can stay there for years. Currently there is no research to indict the manifestation of chronic wasting disease in other animals which pick up the prions in soil.

Dr. Sim and her team have dedicated over two years to understand how hosts and adaptions develop and have so far been very successful. Their findings are being re-affirmed through the testing addition models.

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