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Brain Amyloidoses

Precursor Proteins and the Amyloids of Transmissible and Nontransmissible Dementias: Scrapie—Kuru—CJD Viruses as Infectious Polypeptides or Amyloid-Enhancing Factors

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Biomedical Advances in Aging

Abstract

Kuru and the transmissible virus dementias are in a group of virus-induced slow infections that we have described as subacute spongiform virus encephalopathies because of the strikingly similar histopathological lesions they induce. Scrapie, mink encephalopathy, the chronic wasting disease with spongiform encephalopathy of captive mule deer and of captive elk, and bovine spongiform encephalopathy all appear, from their histopathology, pathogenesis, and the similarities of their infectious agents, to belong to the same group (Gajdusek and Gibbs, 1975; Gajdusek et al., 1965, 1966; Hope et al., 1988; Masters et al., 1981a,b; Wilesmith et al., 1988; Williams and Young, 1980, 1982; Williams et al., 1982). The basic neurocytological lesions in all these diseases are a progressive vacuolation in the dendritic and axonal processes and cell bodies of neurons and, to a lesser extent, in astrocytes and oligodendrocytes; an extensive astroglial hypertrophy and proliferation; and spongiform change or status spongiosis of gray matter and extensive neuronal loss (Beck et al., 1975, 1982; Klatzo et al., 1959).

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  1. Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland, 20892, USA

    D. Carleton Gajdusek & C. Joseph Gibbs Jr.

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  1. The George Washington University Medical Center, Washington, D.C., USA

    Allan L. Goldstein

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Gajdusek, D.C., Gibbs, C.J. (1990). Brain Amyloidoses. In: Goldstein, A.L. (eds) Biomedical Advances in Aging. GWUMC Department of Biochemistry Annual Spring Symposia. Springer, Boston, MA. https://doi.org/10.1007/978-1-4613-0513-2_1

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