Prion as infectious agents and prionopathies
14 important questions on Prion as infectious agents and prionopathies
What are prions? And where do they cause harm?
Group of diseases = prionopathies.
Cause harm in central nervous system => neurodegenerative diseases.
What is the epidemiology of prion diseases?
85% sporadic
15% genetic
When it is genetic; where is the problem?
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Where can we find normal prion proteins? What happens when one prion protein is wrong?
-> signaling mechanims
-> Knock-on effect => domino
What is the pathology behind prion proteins (transmissible spongiform encephalopathies); the cascade? What time does it take?
- One is misfolded
- Knock-on effect
- Autophagy isn't working (deposits amyloid plaques in the brain = donker roze stippen op plaatje)
- Nerve cells get damage + disfunction
- Holes in brain tissue (witte stippen) (= neurodegeneration) -> spongiform changes
- Recruitement of inflammatoiry T-cells (zwarte stippen)
- Personality change -> motor control loosing
- Fatal
Decline can be rapid when the symptoms are found.
But the incubation period can be year.
Which 4 forms of the Creutzfeldt Jakob disease are there?
- Sporadic: 80%, in older people; probably mutagenic event (incidence is growing because of a older world population and improved diagnostics)
- Inherited:15%, autosomal-dominant (50% kans)
- Iatrogenic: by transplantation or donation + human pituitary extracts for growth hormone or infertility treatment (the aberrant protein is stable in heat)
- Variant CJD: discovered in 1996, in young people. Rapid neurological conditions. Caused by a eating bovine (rund) (bovine spongiform encephalopathy BSE)
Where and when were the vCJD found?
Started in 1996.
Sinds 2012 only 2 events.
Concern that there is coming a second wave.
Name different characteristics of PrP(c) and PrP(sc) (7).
How is it called that the disease can be transmitted between closely related species?
What means: scrapie continues to be enzootic in Europe?
How did mad cow disease start?
How does the prion get form the gut to the brain (when it overcomes the species barrier and you eat it for example)?
- How it survives the acidity of the stomach we don't know. But we know that the PrP(sc) is better resistant against
- Better resistant against protease in the gut
- In the distal ileum it goes into the blood stream via the peyer's patches (subepithelial)
- To the lymphoid tissues via the blood. Replication in the B-cells and the follicular dendritic cells.
- To the brain via peripheral nerves and blood.
How does the PrP(sc) go into the membranes of cells?
- Direct exchange = can take a long time = is also what we see = a long latency period without symptoms
- Via the bloodstream = could explain why unconnected regios are affected
How long do patients survive with vCJD?
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