Spontaneous Generation of Infectious Amyloid Nucleants in the Transmissible and Nontransmissible Brain Amyloidoses

  • D. Carleton Gajdusek
Part of the Advances in Research on Neurodegeneration book series (ARN, volume 2)


In recent years we have become aware that the unconventional or atypical slow virus diseases of kuru-Creutzfeldt-Jacob syndrome (CJD) — Gerstmann Sträussler syndrome (GSS) and scrapie-bovine spongiform encephalopathy (BSE) are cerebral amyloidoses (Gajdusek, 1986, 1988a, 1988b, 1989, 1990, 1991a, 1991b, 1991c; Gajdusek and Gibbs, 1990; Gajdusek et al., 1991). As with most amyloidoses, the spontaneous de novo generation of amyloid fibrils is under genetic control, although in many susceptible hosts all individuals are susceptible to even a minimal intracerebral infective dose; thus, for such inoculation there is no genetic control other than the species barrier.


Spontaneous Generation Familial Fatal Insomnia Cerebral Amyloidosis Octapeptide Repeat Transthyretin Amyloidosis 


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© Birkhäuser Boston 1994

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  • D. Carleton Gajdusek

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