Prions

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Prions

caused by conversion of glycoprotein PrPc into an infectious form PrpSc

recent evidence suggests PrPc is involved in regulating cell death

Prpc located on chromosome 20 in humans

1982 Prusiner--Scrapie in sheep caused by prions

spongiform encephalopathies because large vacoules formed in brain\\

Human disease

can be hereditary but not necessarily all the time

fatal familial insomnia

Gerstmann-Straussler-Scheinker syndrome

Creutzfeldt-Jakob disease

kuru

Scrapie was reduced with proteases but not radiation which meant that the infection was a protein