• Jonathan D F Wadsworth, Andrew F Hill, Jonathan A Beck, and John Collinge.
• MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, University College, London, UK.
• Br. Med. Bull. 2003 Jan 1; 66: 241-54.

AbstractWhile rare in humans, the prion diseases have become an area of intense clinical and scientific interest. The recognition that variant Creutzfeldt-Jakob disease is caused by the same prion strain as bovine spongiform encephalopathy in cattle has dramatically highlighted the need for a precise understanding of the molecular biology of human prion diseases. Detailed clinical, pathological and molecular data from a large number of human prion disease cases have shown that distinct abnormal isoforms of prion protein are associated with prion protein gene polymorphism and neuropathological phenotypes. A molecular classification of human prion diseases seems achievable through characterisation of structural differences of the infectious agent itself.

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