Prion Disease Flashcards
in humans, what are prion diseases also known as?
transmissible spongiform encephalopathies
give three examples of human TSEs
Creutzfeldt-Jakob Disease (CJD), Kuru and Fatal Familial Insomnia
what are the two forms of prion protein?
PrPc = natural PrPsc = pathogenic
what is the PrPc protein?
a highly conserved cell surface protein expressed in many tissues, especially neurones
what is the proposed function of the PrPc protein?
thought to be involved in neuronal homeostasis
what is the main structural different between PrPc and PrPsc?
PrPc = mostly a-helical PrPsc = mostly B-sheet
the primary sequence of the PrPc protein is altered on conversion to PrPsc: true or false?
false - PrPsc particles have the exact same primary sequence as PrPc particles
neurodegeneration is prion diseases is thought to occur due to ________ of PrPsc particles
aggregation
PrPsc particles are insoluble in detergents and partially protease-resistant: true or false?
true
why are PrPc and PrPsc particles structurally different?
due to misfolding of the PrPc protein
prion diseases can arise due to… ?
genetic inheritance, infection or sporadically
how does inherited genetic variability within the PRP gene increase susceptibility to prion disease?
mutations increase the likelihood of PrPc protein misfolding
prion disease has a very short incubation period: true or false?
false - prion particles have very long incubation periods
what is kuru?
an incurable, fatal neurological disorder restricted to the Fore people of the Highlands of Papua New Guinea
how many cases of Kuru are currently reported?
11
