Prions

Definition

Prions are infectious proteins that cause neurodegenerative diseases by inducing abnormal folding of normal cellular prion proteins, mainly in the brain. They lack nucleic acids and are resistant to standard methods of decontamination.

5 Must Know Facts For Your Next Test

1. Prions cause diseases such as Creutzfeldt-Jakob disease (CJD) and bovine spongiform encephalopathy (BSE or mad cow disease).
2. They are unique among infectious agents because they contain no genetic material.
3. Prion diseases are invariably fatal and currently untreatable.
4. Transmission can occur through contaminated medical equipment, consumption of infected meat, or inheritance of a mutation in the PRNP gene.
5. Prions induce misfolding of normal proteins into a disease-causing conformation which aggregates and leads to brain damage.

Review Questions

• What makes prions different from other infectious agents?
• Name two diseases caused by prions.
• How can prion diseases be transmitted?

Related terms

Creutzfeldt-Jakob Disease (CJD): A rare, degenerative, fatal brain disorder caused by prions.

Bovine Spongiform Encephalopathy (BSE): A progressive neurological disorder in cattle caused by prions, also known as mad cow disease.

PRNP Gene: The gene that encodes the normal cellular prion protein; mutations can lead to inherited prion diseases.