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Prion Basics

Graphic Illustration of a Prion Protein

What Are Prions?

Prions are distorted versions of normal proteins found in human and animal brain and other tissues. These distorted (‘misfolded’) proteins damage brain cells, leading to fatal dementias akin to human Alzheimer’s and Parkinson’s diseases. One of the amazing things about prions is that, although they are composed only of proteins and contain no genes, they have acquired the ability to be transmitted like other infectious agents such as viruses or bacteria. Best known prion diseases are mad cow disease of cattle and humans, Creutzfeld-Jakob disease of humans, chronic wasting disease of deer and elk, and scrapie of sheep. Better understanding of prions is important in controlling the threat they represent to animals and humans and for what we can learn about the cause and treatment of the major dementia diseases of humans

Woman working on a computer

Why Care About Prion Research?

Prions are known to cause disease among animal and human populations. According to the CDC prions can cause a number of fatal neurological diseases like;

  • Bovine Spongiform Encephalopathy (Mad Cow Disease)
  • Chronic Wasting Disease
  • Scrapie
  • Creutzfeldt-Jakob
  • Transmissible Mink Encephalopathy

Deer doe and fawn in a field

How Are Prion Diseases Transmitted?

Prion diseases can be inherited, acquired by infection, or arise spontaneously without known cause. In humans, prion diseases are predominantly due to sporadic causes and are rarely transmitted between individuals. The most common types of human to human transmission are related of medical procedures involving transfer of tissues from a human with an undiagnosed prion disease. These mainly include recipients of growth hormone therapy and tissue grafts from the fibrous covering of the brain. There have been a few cases of human prion disease related to blood transfusion in the United Kingdom. These cases were related to a human prion disease that was acquired from cattle with bovine prion disease. This is a rare disease in humans that is disappearing due to preventative public health measures. In animals the most important prion diseases (above) result from infectious transmission. In the USA, chronic wasting disease of deer and elk is the most readily transmitted of the prion diseases.

Jeffrey Christiansen of the Prion Research Center looks into a microscope

Research at the Prion Research Center

Researchers at the PRC study many different aspects of prions, including how they form; how they infect cells, tissues and animals; how to detect them and how to treat them. The PRC thinks of these other protein misfolding diseases, like Alzheimer’s, Parkinson’s, Lou Gehrig's and Huntington’s diseases as prion diseases too. The research being conducted at the PRC holds promising significance in the scientific and medical fields and may provide benefits to the research being done on these similar prion diseases.

 

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Prion Research Center
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