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The spongiform encephalopathies are unusual in several respects. Firstly, they are transmissible, and in some cases inheritable. Secondly, variants of these disorders occur in many species and can be transmitted by consumption of infected material; this has led to concern as to the potential risk from eating contaminated animal products. Thirdly, increasing evidence suggests that a ‘prion’ protein is central to their aetiology and pathogenesis, and that no nucleic acid is involved in the infective process. The role of the prion gene and its protein is outlined and proposed as the basis for an improved classification of the transmissible dementias.

Original publication

DOI

10.1192/bjp.158.4.457

Type

Journal article

Journal

British Journal of Psychiatry

Publisher

Royal College of Psychiatrists

Publication Date

04/1991

Volume

158

Pages

457 - 470