NR AAXJ

AU Baker,H.F.; Ridley,R.M.; Crow,T.J.

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

QU British Medical Journal (Clinical Research Edition) 1985 Aug 3; 291(6491): 299-302

PT journal article

AB Marmosets inoculated intracerebrally with brain tissue from a woman with Gerstmann-Sträussler 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-Sträussler 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.

IN Auch das Gerstmann-Sträussler-Syndrom ließ sich mit Gehirnmaterial intracerebral auf Krallenaffen übertragen und erzeugte eine nicht Creutzfeldt-Jakob-Infektionen unterscheidbare spongiforme Encephalopathie.

MH Aged; Animal; Callitrichinae; Creutzfeldt-Jakob Syndrome/*transmission; Female; Fertility; Human; Male; Middle Age; Pedigree; Sex Factors; Slow Virus Diseases/genetics/*transmission

SP englisch

PO England

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