title: Experimental transmission of an autosomal dominant spongiform encephalopathy: does the infectious agent originate in the human genome?
abstract: 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.
mesh_terms: D000368:Aged; D000818:Animals; D002143:Callitrichinae; D007562:Creutzfeldt-Jakob Syndrome / Q000635:transmission*; D005260:Female; D005298:Fertility; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D012737:Sex Factors; D012897:Slow Virus Diseases / Q000235:genetics / Q000635:transmission*