Term
| describe the size and composition of a prion |
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Definition
| small (filterable), glycoprotein particles, no genome or nucleic acids |
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Term
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Definition
| neuronal copper metabolism |
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Term
| why do prions effects take so long to see, 2 reasons |
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Definition
they replicate very slowly
they dont ilict an inflammatory or immune response |
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Term
| what wont kill a prion , why |
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Definition
heat, disinfectats, irradiation, boiling, formaldehyde, autoclaving
lysosome cant break them down
due to beta pleated sheet |
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Term
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Definition
| phenol peridoate, sodium hydroxide, sodium hypochlorite |
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Term
| what are the most common ways of prion transmission (7) |
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Definition
contaminated food (meat)
it sticks very well to metal (surgical insturments)
contaminated blood transfusion
contaminated hormone transfusion
contaminated organ transplant (cornea, dura)
mother-fetus
cross species |
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Term
| what are the general symptoms of a prion infection (10) |
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Definition
neurodegenerative (especially motor): ataxia, gait, paralysis, speech loss, loss of vision, insomnia, memory loss, dementia
spongiform encephalopathy: vacuolization of brain tissue |
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Term
| how can you diagnst a prion infection, why is this challenging (3 statements) |
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Definition
it can be confirmed histologically post mortem
difficult because prions cannot be cultured because there is no immune response
sometimes tonsils show PrPsc via immunoblotting, immunohistochemistry, and immunoassay |
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Term
| how can prion infection be prevented (7) |
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Definition
genetic concouling
stop contaminated blood transfusion
stop contaminated hormone transfusion
avoid contaminated food
avoid canabilism
polyanicic acid
tricyclic acid |
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Term
| what are the 6 ddx that show up when diagnosing a prion disease, give their general MOA |
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Definition
alzheimers: amyloid plaques, tau tangles, loss of neuron connection
parkinsons: loss of dopamine production
lewy bodies: alpha syneuclein protein
picks: tau protein tangles
amytrophic lateral sclerosis: upper and lower motor neuron degeneration
huntingtons: genetically programmed degeneration of nerve cells |
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Term
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Definition
| glycoprotein of unknown function |
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Term
| PrPc: what is its name, where is it located in genome, structure, location |
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Definition
"cellular prion protein:
on chromosome 20
normal potein with alpha helix that is generally linear
located on nerve cell surface |
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Term
| how, structurally, is PrPc different from PrPsc |
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Definition
PrPc is enzyme (protease) susceptible and soluble. it has a alpha helix structure
PrPsc is an abnormal protein that is globular and is made of beta pleated sheets and intracellular fibrils. it is enzyme resistant |
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Term
| how is PrPc made and put in its proper home |
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Definition
| made in ER along with glycophosphatidylinositol which is added onto it, it goes to golgi then is attached to membrane bia glycophosphatidylinositol |
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Term
| PrPsc: AKA, structure, location |
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Definition
"prior protein scaripe"
located in cytoplasm
abnormal protein, beta pleated sheets, globular, with disease causing intracellular fibrils |
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Term
| explain the MOA of a prion infection |
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Definition
1. PrPsc is introduced and contacts PrPc
2. PrPc conformational change to beta pleated sheets via GAG cofactors and raft microdomains
3. PrPc turns into PrPsc and leaves the cell and the cell makes more PrPc to compensate.
4. PrPsc pillages and causes havoc |
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Term
| what facilitates the change of PrPc to PrPsc from alpha helix to beta pleated sheets, what is a possible complication of this process |
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Definition
GAG cofactors and raft microdomains cause a conformational change
this could trigger cell apoptosis |
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Term
| what bad things does PrPsc do |
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Definition
internalized into the cell cytoplasm, losysome, and plasma membrane
causes cell death
accumulates outside of cells making plaques and inhibiting signaling and causing degeneration (neurotoxicity)
travels in lymphatic system on lymphocytes in the blood to the tonsils and spleen |
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Term
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Definition
| prion is ingested in human brains > resides in GI mucosa > travels to lymphatics > travels to CNS > latent for 34-56 years |
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Term
| what are some classic symptoms of kuru (3) |
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Definition
| loss of coordination, shaking limbs, neurological deterioration |
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Term
| what species is kuru transferable to |
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Definition
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Term
| what is the treatment to kuru |
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Definition
| none, resistant to common physical and chemical treatments |
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Term
| crutsfeldt jakob disease - varient: how does one get it, who does it infect, what is the timeline |
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Definition
comes from eating cow meat that has bovine spongiform encephalopathy infecting humans
latency period shorter than normal and even shorter if contracted young |
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Term
| crutsfeldt jakob disease - classic: how does one get it, what causes it, timeline |
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Definition
inherited autosomal dominant disorder of mutated PrP gene.
long latency |
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Term
| bovine spongiform encephalopathy: how does one get it, who gets it |
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Definition
| infects cows that have eaten sheep meat contaminated to scaripe |
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Term
| what are the prion diseases that infect animals, what animal do they targer |
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Definition
bovine spongiform encephalopathy: cows
scaripe: sheep
visna: ?
wasting disease of cervids: deer and elk |
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Term
| what are human infecting prion diseases, in general what causes them |
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Definition
gerstmann strawssler scheinker: mutated PrP gene
fatal familial insomnia: mutated PrP gene
crutsfeldt jakob varient: tainted cow meat
crustfeldt jakob classic: PrP gene mutated |
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