Transmissible spongiform encephalopathies (TSEs)

Definition

Transmissible spongiform encephalopathies (TSEs) are a group of progressive, invariably fatal diseases characterized by spongy degeneration of the brain tissue. They are caused by prions, which are misfolded proteins that induce abnormal folding in normal cellular proteins.

5 Must Know Facts For Your Next Test

1. TSEs are caused by prions, infectious agents composed solely of protein.
2. Common TSEs include Creutzfeldt-Jakob disease (CJD) in humans and Bovine Spongiform Encephalopathy (BSE) in cattle.
3. Prions cause neurodegeneration by inducing abnormal folding of specific normal cellular proteins found in the brain.
4. There is no cure for TSEs, and they are invariably fatal once symptoms appear.
5. Transmission can occur through contaminated food, medical procedures, or inherited genetic mutations.

Review Questions

• What type of infectious agent causes transmissible spongiform encephalopathies?
• Name two common forms of TSEs and the species they affect.
• How do prions cause neurodegeneration in TSEs?

Related terms

Prion:

A misfolded protein that can induce other proteins to also misfold, leading to disease.

Creutzfeldt-Jakob Disease (CJD): A human TSE characterized by rapid neurodegenerative decline and spongy brain tissue.

Bovine Spongiform Encephalopathy (BSE): A TSE affecting cattle, commonly known as 'mad cow disease'.