Chapter 25 - prions Flashcards
What are prions also called?
Transmissible spongiform encephalopathies
Transmissible in TSE is for?
Ingestion, parenteral, iatrogenic, hereditary
Spongiform in TSE is for?
Diseased brain is spongelike, holes in brain
Encephalopathies in TSE is for?
No immune response, no inflammation, no antibodies
Where does the name for prion come from?
Protein infection
Who discovered prions?
Stanley B. Prusiner
What is notable about prions?
They are highly resistant, and affect humans and animals. They do not replicate - they transform. Alpha-helixes turn into beta-pleats, for example
What are the human TSE diseases?
Kuru, Creutzfeldt-Jakob disease (CJD), Variant Creutzfeldt-Jakob disease (vCJD), Gerstmann-Sraussler-Scheinker syndrome (GSS), Fatal familial insomnia (FFI)
Notes about kuru?
Ingestion + parenteral transmission
Causes tremors, ataxia
Common in Fore tribes
Notes about Creutzfeldt-Jakob Disease?
Also called CJD
Sporadic, iatrogenic, hereditary transmission
Causes dementia, jerking, muteness, coma
Growth hormones, corneal transplants, brain electrodes
Notes about Variant Creutzfeldt-Jakob disease?
Also called vCJD
Ingestion
Behavior, psychological, delayed neurological effects
Ingestion of a mad cow
Notes about Gerstmann-Sraussler-Scheinker syndrome?
AKA GSS
Hereditary
Rare autosomal dominant
Ataxia, dementia, coma, deaths
Notes about fatal familial insomnia?
AKA FFI
Hereditary
Rare autosomal dominant
~40 families worldwide
What are the animal TSE diseases?
Scrapie, bovine spongiform encephalopathy (BSE), chronic wasting disease (CWD), transmissible mink encephalopathy, feline spongiform encephalopathy
What animal can get scrapie?
Sheep
