Prions

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Definition

Prions are abnormal, transmissible, pathogenic agents that induce abnormal folding of specific cellular proteins called prion proteins. When prion proteins are abnormally folded, they lead to prion diseases.

Prion diseases, also called transmissible spongiform encephalopathies, are neurodegenerative diseases that can affect humans and a variety of domestic & wild animal species. They are characterized by brain damage caused by abnormal prion proteins.

Requirements for working with prions or prion-infected tissues

Conduct a risk assessment

Most prions affecting humans and/or animals must be handled at a minimum of Biosafety Level 2 (BSL-2) containment. Conduct a risk assessment to determine the appropriate containment level for your work. In your risk assessment, be sure to specifically consider the following:

  • Inactivation: Prions are highly resistant to conventional inactivation procedures. Be sure you understand how you can fully inactivate any prions or prion-contaminated materials you will work with. Consider using disposable lab supplies that can be discarded and incinerated to avoid lengthy decontamination procedures.
  • Fixed versus Unfixed Tissue: Formalin-fixation of tissues containing prions reduces risk of prion exposure but should not be considered to remove this risk entirely.
  • Sharps Usage: Primary hazards are typically associated with accidental skin puncture or accidental ingestion. Use of cut- or puncture-resistant gloves can reduce risk of exposure while working with sharps.
  • Types of Tissue: The highest concentrations of prions are in the central nervous system (brain, spinal cord) and its coverings, but prions have also been found in the spleen, thymus, lymph nodes, and intestines of animals. Consider the types of tissues needed for your experiments and minimize use of tissues that are most likely to harbor prions.

Submit your work for approval by the Institutional Biosafety Committee (IBC)

PIs and Instructors who wish to work with prions or prion-infected tissues must submit a biosafety protocol to the IBC for review and approval before beginning work

  • Submit to the IBC online via iManager. Click here to access the submission system and submission instructions. Fill out the form as completely as possible, and provide any additional pertinent information that may assist the committee in assessing risks associated with your work.
  • The IBC will determine the final biosafety level appropriate for the work proposed. Research should not begin until IBC approval is granted.

Complete required training

Biosafety training is required for all individuals conducting research with prions or prion-infected tissues. Animal Biosafety training may also be required. Find complete biosafety training requirements on the Biosafety Training webpage.

Have a procedure for waste decontamination & disposal

The safest method for ensuring no risk of residual infectivity is to discard materials and destroy them by incineration. Other recommendations for prion inactivation are based on the use of sodium hypochlorite, sodium hydroxide, and/or autoclaving. In most cases, a combination of heat and chemical inactivation processes will be needed. Consult the BMBL or reach out to the Biosafety Office for specific recommended decontamination protocols.