Microbiology Overview Prion Introduction A prion is an infective protein prions are the only known infectious agents that do not contain DNA or RNA when prions infect an organism, they cause normal proteins to misfold and assume the infectious prion form proteins accumulate in the infectious form causing disease proteins remain infectious even after autoclave treatment The prion protein (PrP)is the particular protein that is implicated in all prion diseases normal PrP is denoted PrPC in prion diseases, PrP is misfolded and denoted PrPSc PrPSc forms amyloid aggregates containing β-pleated sheets Prion Diseases in all prion diseases, the accumulation of PrPSc results in spongiform encephalopathy dementia ataxia death Creutzfeldt-Jakob disease rapidly progressive dementia human form of "mad cow disease" ("variant" CJD) Gerstmann-Sträussler-Scheinker syndrome genetic alteration in PrP inherited kuru cannibalism in Papua New Guinea