CHAPTER 29
Prion Diseases
Qingzhong Kong and Richard A. Bessen
Questions
1.
What is a prion disease?
2.
What is the “Protein-only hypothesis”?
3.
What are the major types of human prion diseases based on clinical phenotypes?
4.
What are the major PrPSc types in human prion diseases?
5.
After peripheral infection, prion neuroinvasion is mediated through what cell type?
6.
What is the role of B cells in prion neuroinvasion?
29.
Prion Diseases
Qingzhong Kong and Richard A. Bessen
2
Answers
1.
What is a prion disease?
Prion disease is a family of diverse transmissible neurodegenerative diseases that requires the prion protein for
prion agent replication and pathogenesis.
2.
What is the “Protein-only hypothesis”?
The “Protein-only Hypothesis” proposes that the infectious prion agent is a misfolded form of PrPC devoid of
specific nucleic acids (called PrPSc where “Sc” refers to scrapie) that is capable of self-perpetrating replication
using PrPC as the substrate.
3.
What are the major types of human prion diseases based on clinical phenotypes?
The major types of human prion diseases based on clinical phenotypes include CJD, GSS, Kuru, and Fatal
Insomnia.
4.
What are the major PrPSc types in human prion diseases?
The major PrPSc types in human prion diseases include type 1, type 2 (a and b), and the GSS type.
5.
After peripheral infection, prion neuroinvasion is mediated through what cell type?
Prion neuroinvasion is mediated by FDCs.
6.
What is the role of B cells in prion neuroinvasion?
B cells are required for prion neuroinvasion because they generate lymphotoxins that are required for the
maturation of FDCs.