Journal
BRITISH MEDICAL BULLETIN
Volume 66, Issue -, Pages 241-254Publisher
OXFORD UNIV PRESS
DOI: 10.1093/bmb/66.1.241
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Funding
- MRC [MC_U123160655] Funding Source: UKRI
- Medical Research Council [MC_U123160655] Funding Source: Medline
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While 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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