Transmissible spongiform encephalopathy

Definition

Transmissible spongiform encephalopathy (TSE) is a group of progressive, fatal neurodegenerative diseases caused by prions. These diseases are characterized by brain tissue damage that leads to sponge-like holes in the brain.

5 Must Know Facts For Your Next Test

1. TSEs are caused by misfolded prion proteins that induce other normal proteins to misfold.
2. Common TSEs include Creutzfeldt-Jakob disease in humans, scrapie in sheep, and bovine spongiform encephalopathy (mad cow disease) in cattle.
3. TSEs have long incubation periods but progress rapidly once symptoms appear.
4. Diagnosis of TSEs often involves clinical evaluation, MRI scans, cerebrospinal fluid tests, and sometimes brain biopsies or autopsies.
5. There is currently no cure for TSEs; treatment focuses on alleviating symptoms.

Related terms

Prion:

An infectious protein that can cause neurodegenerative diseases.

Viroid:

A plant pathogen consisting solely of a short strand of circular RNA without a protein coat.

Virusoid:

A type of satellite RNA dependent on a helper virus for replication within host cells.