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.

• Authors: H F Baker, R M Ridley, T J Crow
• Journal: British medical journal (Clinical research ed.) (Br Med J (Clin Res Ed)) Vol. 291 Issue 6491 Pg. 299-302 (Aug 03 1985) ISSN: 0267-0623 [Print] England
• PMID: 3926166 (Publication Type: Journal Article)

Topics

• Aged
• Animals
• Callitrichinae
• Creutzfeldt-Jakob Syndrome (transmission)
• Female
• Fertility
• Humans
• Male
• Middle Aged
• Pedigree
• Sex Factors
• Slow Virus Diseases (genetics, transmission)