Transmissible spongiform encephalopathies (TSEs), or prion diseases, are a group of invariably fatal neurodegenerative diseases of both humans and animals caused, in whole or in part, by an abnormallyfolded form of the normal cellular protein PrP .The normal form of this protein, known as PrPC, undergoes a conformational change during which it acquires a significant amount of β-sheet , producing the pathological form, PrPSc, which is partially protease resistant and insoluble .Prion diseases exist in genetic, sporadic, and acquired forms, all of which are transmissible . The most common human prion disease is Creutzfeld-Jakob Disease (CJD), the variant form (vCJD) of which is likely acquired through ingestion of beef from Bovine Spongiform Encephalopathy (BSE) infected cattle .
Citation: Baydack RS, McKenzie EJ, Robertson C, Booth SA, Jackson M, et al. (2011) Pre-Clinical Changes Observed by Magnetic Resonance Imaging in a Hamster Model of Transmissible Spongiform Encephalopathy: a Potential Biomarker of Prion Infection. J Mol Biomark Diagn 2:120.