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

Br Med J (Clin Res Ed) 1985 Aug;291(6491):299-302

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.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1416561PMC
http://dx.doi.org/10.1136/bmj.291.6491.299DOI Listing
August 1985

Publication Analysis

Top Keywords

gerstmann-straussler syndrome
8
brain tissue
8
marmosets inoculated
8
autosomal dominant
8
typical case
4
tissue typical
4
case creutzfeldt-jakob
4
creutzfeldt-jakob disease
4
disease pedigree
4
disease huntington's
4
huntington's disease
4
indistinguishable marmosets
4
change amyloid
4
spongiform change
4
associated spongiform
4
amyloid deposition
4
deposition developed
4
pedigree patient
4
encephalopathy indistinguishable
4
inoculated brain
4

References

(Supplied by CrossRef)
Creutzfeldt-Jakob disease: patterns of worldwide occurrenc e and the significance of familial and sporadic clustering
Masters, C.L.; Harris, J.O.; Gajdusek, D.C.; CJ, Jr, Gibbs; Bernoulli, C.; Asher, D.M. et al.
Ann Neurol 1979
Survival of Creutzfeldt-Jakob disease virus in formol fixed brain tissue
Gajdusek, D.C.; CJ, Jr, Gibbs et al.
N Engi J Med 1976
Unusual resistance to ionising radiation of the viruses of kuru, Creutzfeldt-Jakob
CJ, Jr, Gibbs; DC, Gaidusek; R, Latarjet et al.
1978
The familial occurrence of CreutzfeldtJakob disease and Alzheimer's disease
Masters, C.L.; Gajdusek, D.C.; CJ, Jr, Gibbs et al.
Brain 1981
Creuzfeldt-Jakob disease virus isolations from the Gerstmann-Straussler syndrome
Masters, C.L.; Gaidusek, D.C.; Gibbs, C.J. et al.
Brain 1981
J7ournal of the
antimoniate et al.
National Cancer Institute 1974
Antiviral compound effective against experimental scrapie
Kimberlin, R.H.; Walker, C.A. et al.
Lancet 1979

Similar Publications