Prions and Prion Diseases Flashcards
What is the technical name for prion diseases?
Transmissable spongiform encephalopathies
What species does scrapie infect and what type of disease is it?
Sheep, it is a prion disease
What causes kuru?
Consumption of infected human tissue
What histological change charactizes scrapie, kuru, and Creutzfelt-Jacob?
Spongiform changes of the cerebral gray matter (also loss of neurons and reactive gliosis)
What is the normal protein from which diseased prion proteins are derived?
PrPc
What are the three forms of prion diseases?
Sporadic (most common), Genetic (next most common), Acquired
What is the incidence of CJD in the world population?
1 to 2 per million people
What type of disease is Gerstmann-Straussler-Sheinker disease and what is its incidence?
A genetic prion disease, occurs in one per ten million to one per hundred million
What is thought to be the cause of vCJD?
Eating cow infected with Bovine Spongiform Encephalopathy (BSE)
Are human prion diseases infectious, contagious, or both?
Only infectious. This means the prions that cause them cause disease, but the disease is not spread from human to human
What two mutations are the most common causes of Gerstmann-Straussler-Scheinker disease and what gene are these mutations in?
In gene for prion protein, mutations P102L and A117V
What type of disease is fatal familial insomnia?
It is an inherited prion disease (mutation D178N in prion protein)
What are the two findings, either of which is diagnostic of a prion disease?
Proteinase K resistant form of prion protein in brain tissue, or prion disease causing mutation in prion protein gene
What new disease may be an exception to the diagnostic rule of prion diseases?
Protease-sensitive prionopathy
What genetic polymorphism may influence susceptibility to prion diseases?
Codon 129 of the prion protein gene
