Prion diseases are a group of infectious neurodegenerative diseases with an entirely novel mechanism of transmission, involving a protein-only infectious agent that propagates the disease by transmitting protein conformational changes.
The disease results from extensive and progressive brain degeneration.
What are the diseases caused by prions?
Identified Prion Diseases
- Creutzfeldt-Jakob Disease (CJD)
- Variant Creutzfeldt-Jakob Disease (vCJD)
- Gerstmann-Straussler-Scheinker Syndrome.
- Fatal Familial Insomnia.
How do prions cause neurodegenerative disease?
Brain-wasting proteins called prions kill neurons by shortening the dendritic spines that the cells use to transmit signals to each other. Prions are infectious and cause neurodegenerative diseases such as scrapie in animals and Creutzfeldt–Jakob disease in humans.
How does prion disease affect the nervous system?
When prions reach a certain number, disease results. Prions never change back into normal PrPC. PrPC is present in all cells of the body but has a high concentration in the brain. Consequently, most prion diseases affect the nervous system predominantly or exclusively.
Is als a prion disease?
The buildup of proteins in the brain and spinal cord is a hallmark of ALS. And once inside these neurons, these misfolded proteins can trigger the aggregation of the cells’ own copies of the enzyme. These results suggest that ALS may be similar to prion diseases such as the brain disorder Creutzfeldt-Jakob disease.