2 Flashcards
what are prions?
non conventional infectious agents found in brain tissue (infectious abnormal proteins)
abnormal print form of protein is PrP^Sc
**also these lil suckers are hard to kill
how are those lil prion guys infectious?
they create complexes/crystals that the body can’t break down so you end up with a spongy brain
spongiform encephalopathies
(spongy brain duh)
detectable by the presence of amyloid rods
associated with long incubation periods culminating in death
scrapie
(sheep/goats spongy brain)
infected animals **scrape themselves against fences and and walls until raw
they gradually lose motor control and die (bc brain dying)
bovine spongiform encephalopathy (BSE)
“mad cow disease” (cow spongy brain)
Great Britain outbreak began in 1986 from an unknown origin
chronic wasting disease
(deer/elk in Montana)
Kuru disease
from south pacific (affects humans)
- associated with cannibalism (chowin’ down on that neural tissue)
- dementia
- progressive insomnia
- death
**also a spongy brain thing
creutzfeldt-jakob disease (CJD)
spongy brain thing that is a genetic fluke (not from eating infected neural tissue)
~1-1.5 cases per/million in the US
happens in families
variant form (vCJD) - from eating nervous tissue from a BSE infected animal
other spongy brain diseases of humans
- fatal familial insomnia
2. gerstmann-straussler-scheinker disease (GSS)
how are prions induced?
horizontal transfer (eating tissue, getting transplant)
vertical transfer (genetic inheritance)
spontaneous mutation (random)
are prions distinct diseases?
idk bc similar to alzheimer’s
what is the immune response to prions?
bc they are your proteins
can animal prion diseases cause human neurological diseases?
some probably
some not yet
we need to be ready
what are prions more resistant than?
endospores
