Skip to main content
British Medical Journal (Clinical Research Ed.) logoLink to British Medical Journal (Clinical Research Ed.)
. 1985 Aug 3;291(6491):299–302. doi: 10.1136/bmj.291.6491.299

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

H F Baker, R M Ridley, T J Crow
PMCID: PMC1416561  PMID: 3926166

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.

Full text

PDF
299

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Adam J., Crow T. J., Duchen L. W., Scaravilli F., Spokes E. Familial cerebral amyloidosis and spongiform encephalopathy. J Neurol Neurosurg Psychiatry. 1982 Jan;45(1):37–45. doi: 10.1136/jnnp.45.1.37. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. Bernoulli C., Siegfried J., Baumgartner G., Regli F., Rabinowicz T., Gajdusek D. C., Gibbs C. J., Jr Danger of accidental person-to-person transmission of Creutzfeldt-Jakob disease by surgery. Lancet. 1977 Feb 26;1(8009):478–479. doi: 10.1016/s0140-6736(77)91958-4. [DOI] [PubMed] [Google Scholar]
  3. Brown P., Hooks J., Roos R., Gajdusek D. C., Gibbs C. J., Jr Attempt to identify the agent for Creutzfeldt-Jakob disease by CF antibody relationship to known viruses. Nat New Biol. 1972 Feb 2;235(57):149–152. doi: 10.1038/newbio235149a0. [DOI] [PubMed] [Google Scholar]
  4. Gajdusek D. C., Gibbs C. J., Jr Letter: Survival of Creutzfeldt-Jakob-disease virus in formol-fixed brain tissue. N Engl J Med. 1976 Mar 4;294(10):553–553. doi: 10.1056/nejm197603042941015. [DOI] [PubMed] [Google Scholar]
  5. Gibbs C. J., Jr, Gajdusek D. C. Experimental subacute spongiform virus encephalopathies in primates and other laboratory animals. Science. 1973 Oct 5;182(4107):67–68. doi: 10.1126/science.182.4107.67. [DOI] [PubMed] [Google Scholar]
  6. Gibbs C. J., Jr, Gajdusek D. C., Latarjet R. Unusual resistance to ionizing radiation of the viruses of kuru, Creutzfeldt-Jakob disease, and scrapie. Proc Natl Acad Sci U S A. 1978 Dec;75(12):6268–6270. doi: 10.1073/pnas.75.12.6268. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. Jasmin C., Chermann J. C., Herve G., Teze A., Souchay P., Boy-Loustau C., Raybaud N., Sinoussi F., Raynaud M. In vivo inhibition of murine leukemia and sarcoma viruses by the heteropolyanion 5-tungsto-2-antimoniate. J Natl Cancer Inst. 1974 Aug;53(2):469–474. doi: 10.1093/jnci/53.2.469. [DOI] [PubMed] [Google Scholar]
  8. Kimberlin R. H., Walker C. A. Antiviral compound effective against experimental scrapie. Lancet. 1979 Sep 15;2(8142):591–592. doi: 10.1016/s0140-6736(79)91654-4. [DOI] [PubMed] [Google Scholar]
  9. Masters C. L., Gajdusek D. C., Gibbs C. J., Jr Creutzfeldt-Jakob disease virus isolations from the Gerstmann-Sträussler syndrome with an analysis of the various forms of amyloid plaque deposition in the virus-induced spongiform encephalopathies. Brain. 1981 Sep;104(3):559–588. doi: 10.1093/brain/104.3.559. [DOI] [PubMed] [Google Scholar]
  10. Masters C. L., Gajdusek D. C., Gibbs C. J., Jr The familial occurrence of Creutzfeldt-Jakob disease and Alzheimer's disease. Brain. 1981 Sep;104(3):535–558. doi: 10.1093/brain/104.3.535. [DOI] [PubMed] [Google Scholar]
  11. Masters C. L., Harris J. O., Gajdusek D. C., Gibbs C. J., Jr, Bernoulli C., Asher D. M. Creutzfeldt-Jakob disease: patterns of worldwide occurrence and the significance of familial and sporadic clustering. Ann Neurol. 1979 Feb;5(2):177–188. doi: 10.1002/ana.410050212. [DOI] [PubMed] [Google Scholar]
  12. Prusiner S. B. Novel proteinaceous infectious particles cause scrapie. Science. 1982 Apr 9;216(4542):136–144. doi: 10.1126/science.6801762. [DOI] [PubMed] [Google Scholar]
  13. Rosenthal N. P., Keesey J., Crandall B., Brown W. J. Familial neurological disease associated with spongiform encephalopathy. Arch Neurol. 1976 Apr;33(4):252–259. doi: 10.1001/archneur.1976.00500040036005. [DOI] [PubMed] [Google Scholar]
  14. Werner G. H., Jasmin C., Chermann J. C. Effect of ammonium 5-tungsto-2-antimoniate on encephalomyocarditis and vesicular stomatitis virus infections in mice. J Gen Virol. 1976 Apr;31(1):59–64. doi: 10.1099/0022-1317-31-1-59. [DOI] [PubMed] [Google Scholar]

Articles from British Medical Journal (Clinical research ed.) are provided here courtesy of BMJ Publishing Group

RESOURCES