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The University of Southampton
Environmental Healthcare UnitResearch


Infectious prions are misfolded, β-sheet isoforms of the host encoded cellular prion (PrPc) protein that are the causative agents in transmissible spongiform encephalopathies (TSE) including bovine spongiform encephalopathy and Creutzfeldt–Jakob disease.

Comparison of PrPC and PrPSc

Transmissible Spongiform Encephalopathies (TSE’s) or prion diseases are a group of rare, transmissible, and fatal, brain diseases, of which probably the most well-known are Bovine Spongiform Encephalopathy (BSE) in cattle and Creutzfeldt-Jakob Disease (CJD) in humans. This group of disorders have been coined prion diseases due to the widely held belief that the main protagonist of the disease is a naturally occurring, but mis-folded, protein; and it is this proteinaceous infectious particle that has provided the basis for the term prion.

CJD itself can be split into three distinct sub-types:

At the University of Southampton work on the elimination of iatrogenic CJD has been initiated.

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