Experimental transmission of an autosomal dominant spongiform encephalopathy: does the infectious agent originate in the human genome?

Marmosets inoculated intracerebrally with brain tissue from a woman with Gerstmann-Straussler syndrome (an autosomal dominant dementia associated with spongiform change and amyloid deposition) developed an encephalopathy indistinguishable from that seen in marmosets inoculated with brain tissue from a typical case of Creutzfeldt-Jakob disease. As in Huntington's disease, in the pedigree of the patient with Gerstmann-Straussler syndrome women who subsequently developed the illness had increased fecundity. The pathogen in human transmissible dementia may arise from a sequence (which itself sometimes confers a selective advantage) located within the human genome.
AuthorsH F Baker, R M Ridley, T J Crow
JournalBritish medical journal (Clinical research ed.) (Br Med J (Clin Res Ed)) Vol. 291 Issue 6491 Pg. 299-302 (Aug 3 1985) ISSN: 0267-0623 [Print] ENGLAND
PMID3926166 (Publication Type: Journal Article)
  • Aged
  • Animals
  • Callitrichinae
  • Creutzfeldt-Jakob Syndrome (transmission)
  • Female
  • Fertility
  • Humans
  • Male
  • Middle Aged
  • Pedigree
  • Sex Factors
  • Slow Virus Diseases (genetics, transmission)

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