Normal prion protein and pathogenic prion protein (abnormally folded)

Prions are believed to be the causative agents of transmissible spongiform encephalitis (TSE). TSEs are a group of neurodegenerative disorders that affect humans and some animals. Although prions are a protein normally found in brain and are abundant there, they have a nasty counterpart that are abnormally folded. This abnormal conformation allows the prion protein to become infectious. These abnormal prions kill neurons leaving fluid filled spaces behind and hence the use of the word spongiform to describe the anatomy. The abbreviation PrP-sen refers to the normal prion and PrP-res refers to the disease causing prion. The abnormally folded prion is thought to cause normal prions to fold abnormally creating a chain reaction.

Normal prion function is not completely understood at this time. Prions are located on the surface of neurons but are also found in other tissues. Early research suggests that prions play a role in protecting neurons and helping them respond to oxygen deficiencies. Prion TSEs are usually rapidly progressive and at this time fatal.

Prions are linked to Creutzfeldt-Jakob disease (CJD, human disease), Kuru (New Guinea, human), Bovine Spongiform Encephalopathy (BSE, cow), Scrapie (sheep, goat), and Ungulate Spongiform Encephalopathy (deer) to mention a few. Studies suggest that point mutations of the human prion gene, that lead to amino acid substitutions, convert a normal prion into an abnormally folded prion leading to CJD.

Abnormal prion proteins are very resistant to disinfectants, heat, UV and ionizing radiation as well as formalin. Nevertheless, they can be denatured by heat, chemicals, combinations of heat and chemicals, pressure and time. Prions have been reported to denature when incinerated at 900 °F for 4 hours. Using an autoclave, prions can be denatured when using moist heat at 270 °F at 21 psi for 90 minutes.

A study done at the University of Alberta back in early 2012 reports that the prion is susceptible to molecular ozone. This means that this may be a simple solution to decontaminating medical and dental surgical instruments, waste products from slaughterhouses, treating water, and waste water. Dr. Neumann reports “It is possible to take a medical instrument, put it in an ozone bath and very quickly destroy 99.99 percent of the prions that are there.” At this point, they need to actually infect an animal with the treated prion to validate their findings.

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For more information on prion deactivation: