02a: Protein Folding Diseases Flashcards
Diseases of protein folding are formally called:
Proteopathies
Neurodegenerative diseases typically due to:
Protein aggregation (accumulation of misfolded protein)
Protein aggregation, leading to disease, happens (inside/outside) neurons.
Either!
Do prions have DNA or RNA?
Neither
Prions are what type of molecules?
Naked protein molecules
How are prion diseases acquired?
- Infection
- Genetically inherited
- Sporadically
Prion diseases develop (slower/quicker/same as) compared to other neuro degenerative diseases.
Quicker
Scrapie is a (X) disease found in:
X = prion
Sheep
BSE or (X) disease is a (Y) disease found in:
X = bovine spongiform encephalopathy (Mad Cow)
Y = prion
Cows
CWD or (X) disease is a (Y) disease found in:
X = chronic wasting
Y = prion
Deer and elk
CJD or (X) disease is a (Y) disease found in:
X = Creutzfeldt-Jakob Disease
Y = prion
Humans
Kuru disease is a (Y) disease found in:
Y = prion
Humans
Which clinical features are found in prion diseases, but not other neuro degenerative diseases like AD?
Muscle symptoms (ataxia, tremors, myoclonus)
T/F: prion diseases, unlike AD, don’t cause dementia.
False
Describe neuropathology of prion diseases.
- Spongiform change in brain
2. Amyloid plaques
Kuru has similar pathology to which other prion disease?
Scrapie
Which prion diseases are familial (inherited)?
CJD, GSS, FFI
Were the first BSE cases inherited, infectious, or sporadic?
Infectious (from sheep scrapie) or sporadic
How were the first cases of CJD acquired?
Infection by eating contaminated beef
What’s a PrPc? Function?
Normal cell-surface protein (unknown function)
Is PrPc infectious?
No
T/F: PrPc is monomeric.
True
T/F: PrPsc is monomeric.
False - aggregated
What’s the composition of secondary structure in PrPc?
3% beta sheets
40% alpha helices
