Research Article

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

Br Med J (Clin Res Ed) 1985; 291 doi: (Published 03 August 1985) Cite this as: Br Med J (Clin Res Ed) 1985;291:299
  1. H F Baker,
  2. R M Ridley,
  3. T J Crow


    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.