Prevalence in Britain of abnormal prion protein in human appendices before and after exposure to the cattle BSE epizootic

O Noel Gill, Yvonne Spencer, Angela Richard-Loendt, Carole Kelly, David Brown, Katy Sinka, Nick Andrews, Reza Dabaghian, Marion Simmons, Philip Edwards, Peter Bellerby, David J Everest, Mark McCall, Linda M McCardle, Jacqueline Linehan, Simon Mead, David A Hilton, James W Ironside, Sebastian Brandner

Research output: Contribution to journalArticlepeer-review


Widespread dietary exposure of the population of Britain to bovine spongiform encephalopathy (BSE) prions in the 1980s and 1990s led to the emergence of variant Creutzfeldt-Jakob Disease (vCJD) in humans. Two previous appendectomy sample surveys (Appendix-1 and -2) estimated the prevalence of abnormal prion protein (PrP) in the British population exposed to BSE to be 237 per million and 493 per million, respectively. The Appendix-3 survey was recommended to measure the prevalence of abnormal PrP in population groups thought to have been unexposed to BSE. Immunohistochemistry for abnormal PrP was performed on 29,516 samples from appendices removed between 1962 and 1979 from persons born between 1891 through 1965, and from those born after 1996 that had been operated on from 2000 through 2014. Seven appendices were positive for abnormal PrP, of which two were from the pre-BSE-exposure era and five from the post BSE-exposure period. None of the seven positive samples were from appendices removed before 1977, or in patients born after 2000 and none came from individuals diagnosed with vCJD. There was no statistical difference in the prevalence of abnormal PrP across birth and exposure cohorts. Two interpretations are possible. Either there is a low background prevalence of abnormal PrP in human lymphoid tissues that may not progress to vCJD. Alternatively, all positive specimens are attributable to BSE exposure, a finding that would necessitate human exposure having begun in the late 1970s and continuing through the late 1990s.

Original languageEnglish
Pages (from-to)965-976
Number of pages12
JournalActa Neuropathologica
Publication statusE-pub ahead of print - 30 Mar 2020


Dive into the research topics of 'Prevalence in Britain of abnormal prion protein in human appendices before and after exposure to the cattle BSE epizootic'. Together they form a unique fingerprint.

Cite this