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MPBD: Prion diseases and prion-like mechanisms summary
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MPBD: Prion Disease and Prion-like MechanismsSporadic: 4) Dementia, during which the patient becomes
Creutzfeldt-Jakob disease (CJD) unresponsive or mute (6 months)
Genetic: This disease is caused by a pathology in the
• Familial Creutzfeldt-Jakob disease (fCJD) thalamus. Normal sleep stages go from
• Fatal familial insomnia (sFI) awakening to REM sleep to stage 4 sleep, but in
fatal familial insomnia, the sleep does not go
Acquired: beyond stage 1.
• Kuru
• Iatrogenic Creutzfeldt-Jakob disease (iCJD)
• Variant Creutzfeldt-Jakob disease (vCJD)
Acquired Prion disease pathology
Prions (Prion protein, PrP) are misfolded
proteins that can transmit their misfolded
shape onto normal variants of the same They also found a protective gene against prion
protein. You can get this disease from eating diseases. They found this gene in poor people in
contaminated meat for example. What is New Guinea after the Kuru pandemic. Kuru is a
typical for the pathology of this disease is that disease that is caused by an infectious prion
prion proteins outside of the cells surround found in contaminated human brain tissue. These
large vacuoles, this is called spongiform people were cannibals and ate the brains of their
degeneration. It is the same ancestors which made them sick. But the people
principal as the other diseases. that survived this have a protective heterozygous
You have an unfolded protein allele. To test this they introduced this gene into
that gets very sticky. More a knock-out mouse and saw that when they have
Proteins are going to stick this gene then the progression of the prion
together and form aggregates. But what is disease was very minimal. This means that the
very specific for prion diseases is that prion disease is dependent on the genotype of the
normally the protein has an alpha-helical host. (Left you see the wildtype mouse and on the
form, but when it’s misfolded it gets a prion, right, you see the mouse with the newly
beta-sheet shape and transfers this form to the introduced gene.)
other normal proteins.
Potential sources you can get this disease from
are the food industry, organ transplants, drugs
of human origin, blood banks and plasma
products, and brain surgery.
Long-term graft viability in Parkinson’s with
Genetic Prion disease pathology dopaminergic markers that were still viable after
Mutations in the prion genes cause disease. 14 years. What they found in this tissue is that it
also contained Lewy bodies and ubiquitin-positive
Fatal familial insomnia disease stages: material. This means that some of the graft has
1) Increasing insomnia, resulting in panic taken up the pathology of the host tissue, this
attacks, paranoia and phobias (4 months) sounds logical but is very shocking. The graft is
2) Hallucinations and panic attacks (5 from embryos and should not get this pathology,
months) so is this a prion disease? They investigated this
3) Complete inability to sleep is followed by and discovered that all these aggregated proteins
rapid loss of weight (3 months) have some sort of prion-like behaviour. All of
them can recruit their normal
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