Summary
Abnormal fibrillary structures, designated “scrapie-associated fibrils” (SAF), have been observed using negative stain techniques in subfractions of brains from scrapie-affected animals. SAF have been observed in all combinations of strain of scrapie agent and strain or species of host examined, regardless of their histopathology, in particular the presence or absence of amyloid plaques. SAF consist either of two or four filaments. They are morphologically dissimilar to the normal brain fibrils — microtubules, neurofilaments, glial filaments, and F actin. However, SAF do bear a resemblance to amyloid.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Amos LA (1979) Structure of microtubules. In: Roberts K, Hyams JS (eds) Microtubules. Academic Press, London, pp 1–64
Boeré H, Ruinen L, Scholten JH (1965) Electron-microscopic studies on the fibrillar component of human splenic amyloid. J Lab Clin Med 66:943–951
Chandler RL (1963) Experimental scrapie in the mouse. Res Vet Sci 4:276–285
Cohen RS, Blomberg F, Berzins K, Siekevitz P (1977) The structure of postsynaptic densities isolated from dog cerebral cortex. I. Overall morphology and protein composition. J Cell Biol 74:181–203
Dickinson AG, Fraser H (1977) The pathogenesis of scrapie in inbred mice: an assessment of host control and response, involving many strains of agent. In: ter Meulen V, Katz M (eds) Slow virus infections of the central nervous system. Springer, New York, pp 3–13
Dickinson AG, Fraser H, Bruce M (1979) Animal models for the dementias. In: Glen AIM, Whalley LJ (eds) Alzheimer's disease. Early recognition of potentially reversible deficits. Churchill Livingstone, Edinburgh London New York, pp 42–45
Flament-Durand J, Couck AM (1979) Spongiform alteration in brain biopsies of presenile dementia. Acta Neuropathol (Berl) 46: 159–162
Forer A (1978) Electron microscopy of actin. In: Hayat MA (ed) Principles and techniques of electron microscopy, vol 9. van Nostrand Reinhold, New York, pp 126–174
Fraser H (1976) The pathology of natural and experimental scrapie. In: Kimberlin RH (ed) Slow virus diseases of animals and man. North Holland, Amsterdam New York, pp 209–241
Fraser H (1980) Neuropathology of Scrapie: The precision of the lesions and their diversity. In: Prusiner SB, Hadlow WJ (eds) Slow transmissible diseases of the nervous system, vol 1. Academic Press, New York, pp 387–406
Glenner GG (1980) Amyloid deposits and amyloidosis. N Engl J Med 302:1283–1292
Glenner GG, Canes ED, Bladen HA, Linke RP, Termine JD (1974) \-pleated sheet fibrils. A comparison of native amyloid with synthetic protein fibrils. J Histochem Cytochem 22:1141–1158
Glenner GG, Page D, Iserski C, Harada M, Cuatrecasas P, Canes ED, DeLellis RA, Bladen HA, Keiser HR (1971) Murine amyloid fibril protein: Isolation purification, and characterization. J Histochem Cytochem 19:16–28
Iqbal K, Wisniewski H (1979) Neurofilament proteins: Effect of ionic strength and a calcium chelator. Trans Am Soc Neurochem 10:168
Kim H, Binder LI, Rosenbaum JL (1979) The periodic association of MAP2 with brain microtubules in vitro. J Cell Biol 80:266–276
Kimberlin RH (1976) Biochemical and behavioral changes in scrapie. In: Kimberlin RH (ed) Slow virus diseases of animals and man. North Holland, Amsterdam New York, pp 307–323
Kimberlin RH, Walker CA (1977) Characteristics of a short incubation model of scrapie in the golden hamster. J Gen Virol 34:295–304
Krishnan N, Kaiserman-Abramof IR, Lasek RJ (1979) Helical substructure of neurofilaments isolated fromMyxicola and squid giant axons. J Cell Biol 82:323–335
Oppenheimer DR (1975) Pathology of transmissible and degenerative diseases of the nervous system. In: Illis LS (ed) Viral diseases of the central nervous system. Williams and Wilkins, Baltimore, pp 161–174
Outram GW (1976) The pathogenesis of scrapie in mice. In: Kimberlin RH (ed) Slow virus diseases of animals and man. North Holland, Amsterdam New York, pp 325–357
Rueger DC, Huston JS, Dahl D, Bignami A (1979) Formation of 100 Å filaments from purified glial fibrillary acid protein in vitro. J Mol Biol 135:53–68
Schlaepfer WW (1977) Immunological and ultrastructural studies of neurofilaments isolated from rat peripheral nerve. J Cell Biol 74:226–240
Shelanski ML, Albert S, Devries GH, Norton WT (1971) Isolation of filaments from brain. Science 174:1242–1245
Shelanski ML, Gaskin F, Cantor CR (1973) Microtubule assembly in the absence of added nucleotides. Proc Natl Acad Sci USA 70:765–768
Spudich JA, Watt S (1971) The regulation of rabbit skeletal muscle contraction. J Biol Chem 246:4866–4871
Spurr AR (1969) A low viscosity epoxy resin embedding medium for electron microscopy. J Ultrastruct Res 26:31–43
Whittaker VP (1969) The synaptosome. In: Lajtha A (ed) Handbook of neurochemistry, vol 2. Plenum, New York, pp 327–364
Wisniewski HM (1979) Neurofibrillary and synaptic pathology in senile dementias of the Alzheimer's type. In: Glen AIM, Whalley LJ (eds) Alzheimer's disease. Early recognition of potentially reversible deficits. Churchill Livingstone, Edinburgh London New York, pp 7–16
Wisniewski HM, Bruce ME, Fraser H (1975) Infectious etiology of neuritic (senile) plaques in mice. Science 190:1108–1110
Wisniewski HM, Terry RD (1973) Morphology of the aging brain, human and animal. Prog Brain Res 40:167–186