PrPsc

Definition

PrPsc is an abnormal, pathogenic form of the prion protein. It is responsible for causing transmissible spongiform encephalopathies (TSEs) in humans and animals.

5 Must Know Facts For Your Next Test

1. PrPsc stands for protease-resistant prion protein scrapie.
2. It converts normal cellular prion proteins (PrPc) into its abnormal form through a conformational change.
3. PrPsc accumulates in brain tissue, leading to neurodegenerative diseases such as Creutzfeldt-Jakob disease (CJD) and scrapie.
4. It is highly resistant to standard decontamination methods, including heat and chemical treatments.
5. The presence of PrPsc can be confirmed through techniques like Western blotting or immunohistochemistry.

Review Questions

• What is the primary function of PrPsc in prion diseases?
• How does PrPsc differ from the normal cellular prion protein (PrPc)?
• Which neurodegenerative diseases are associated with the accumulation of PrPsc?

Related terms

PrPc:

The normal cellular form of the prion protein that can be converted into PrPsc.

Transmissible Spongiform Encephalopathies (TSEs): A group of progressive conditions that affect the brain and nervous system in humans and animals.

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