Experimental transmission of an autosomal dominant spongiform encephalopathy: does the infectious agent originate in the human genome?. Issue 6491 (3rd August 1985)

• Record Type: Journal Article
• Authors: Baker, H F; Ridley, R M; Crow, T J
• Is Part Of: BMJ. Volume 291:Issue 6491(1985)
• Page Start: 299
• Page End: 302
• Publication Date: 1985-08-03
• Subjects: Medicine -- Periodicals; Medicine -- Periodicals; Medicine; Periodicals; 610
• DOI: 10.1136/bmj.291.6491.299
• Languages: English
• ISSNs: 0007-1447

Abstract

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.