Prions Flashcards
What does it mean to be infectious? List 5 keywords.
Propagation. Pathogenic. Opportunistic. Evasive. More available. Communicable. Transmissable. Contagious.
Give a short description of a prion.
They are proteinaceous infectious particles with no nucleic acid. They propogate but don’t replicate. Transmitted infection by misfolding and implicated in neurodegenerative diseases.
Describe PrPC
Encoded by host. Membrane glycolipoprotein. Expressed on neurons, lymphocytes, and folicular DCs. May be useful in immune syst, signals, copper binding, and apoptosis.
Describe PrPSc
Diseased PrP. Aggregated form, partially protease resistant. Variants.
Describe PrP protein structure/domains.
Signal peptide for ER entry. GPI anchored to membrane. Cleavage site on N terminus.
Mutations in PrP gene are associated with _____
Disease outcomes.
What theory ties PrP with Copper?
Theory that states PrPc contributes to copper homeostasis at neural pre-synaptic membrane. Acts as copper scavenger.
What are the two promosed models for prion propagation?
Refolding model: good is converted by bad.
Seeding model: accumulation of bad which splits.
What is the protein only hypothesis?
States that PrPsc is from PrP c. Unanimously believed.
What kind of CJD can be caused by medical procedure?
Iatrogenic CJD
What are TSE symptoms?
Rapid dementia, visual/cerebellar signs, mutism, frontal/cerebellar injury likeness
Key infected tissues of TSEs are ___
CNS and PNS, spleen, lymph nodes, nasal mucosa, intestine, muscles, and eyes.
Describe the neuropathology of TSEs.
It depends on the disease as to where it will be expressed in the brain.
What is the incubation range of TSEs?
20-50 years. 85% of cases have death within a year of symptom presentation.
