Molecular Neurobiology

, Volume 8, Issue 1, pp 1–13

Spontaneous generation of infectious nucleating amyloids in the transmissible and nontransmissible cerebral amyloidoses

  • D. Carleton Gajdusek

DOI: 10.1007/BF02778003

Cite this article as:
Gajdusek, D.C. Mol Neurobiol (1994) 8: 1. doi:10.1007/BF02778003


The unconventional viruses of the transmissible subacute spongiform encephalopathies (kuru-CJD-GSS-FFI-scrapie-BSE) are nucleants spontaneously generated from host precursor proteins altered to β-pleated sheet configuration that polymerize into insoluble infectious amyloid fibrils. Thede novo conversion to infectious amyloids is facilitated or accelerated by many different point mutations causing amino acid changes, a stop codon, or octapeptide inserts that increase the likelihood of spontaneous conversion to infectious configuration by many orders of magnitude. Similar nucleating induction of configurational change to amyloid probably occurs in other amyloidoses of brain and in systemic amyloidoses. Thus, all amyloids, particularly so-called fibrillar amyloid enhancing factors, may be considered to be infectious scrapie-like agents. These events probably occur extracellularly, thus we are attempting to reproduce them in vitro, even from synthetic polypeptides.

Index Entries

Nucleation infectious amyloids spontaneous generation of infectious proteins amyloidoses, of brain, nontransmissible amyloidoses, of brain transmissible Creutzfeldt-Jacob disease Gerstmann-Sträussler-Scheinker disease familial fatal insomnia scrapie bovine spongiform encephalopathy amyloidoses familial amyloidotic polyneuropathy Alzheimer’s disease protein configuration 

Copyright information

© Humana Press Inc 1994

Authors and Affiliations

  • D. Carleton Gajdusek
    • 1
  1. 1.Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and StrokeNational Institutes of HealthBethesda

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