Unclassified: Spongiform Encephalopathies

Merz, Patricia A.; Wı́sniewski, Henryk M.

doi:10.1007/978-1-4612-3900-0_42

Cited by 2 publications

(1 citation statement)

References 179 publications

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“…Based on these ultrastructural characteristics, the fibrils have been reported repeatedly to be different from a m y l~i d .~~. 68 Attempts to stain the fibrils with Congo red dye have yielded negative result^;^^^ 6 7 * 68 however, even a positive result would have been uninterpretable because of impurities in the extracts.…”

Section: Prion Rods and Filaments Are Amyloidmentioning

confidence: 97%

Prions are novel infectious pathogens causing scrapie and creutzfeldt—Jakob disease

Prusiner

1986

BioEssays

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SummaryScrapie and Creutzfeldt-Jakob disease (CJD) are caused by prions, which appear to be different from both viruses and viroids. Prions contain protein which is required for infectivity, but no nucleic acid has been found within them. Prion proteins are encoded by a cellular gene and not by a nucleic acid within the infectious prion particle. A cellular homologue of the prion protein has been identijied. The role of this homologue in metabolism is unknown. Prion proteins, but not the cellular homologue, aggregate into rod-shaped particles that are histochemically and ultrastructurally identical to amyloid. Extracellular collections of prion proteins form amyloid plaques in scrapie-and CJD-infected rodent brains as well as CJD-infected human brains. Within the plaques, prion proteins assemble to form amyloidJlaments. Elucidating the molecular dtgerences between the prion protein and its cellular homorogue may be important in understanding the chemical structure and replication of prions. Animal and Human Prion DiseasesIn developing models for the study of degenerative neurologic diseases, we have focused our attention on the study of scrapie, a degenerative neurologic disorder of sheep and goats. Scrapie occurs after a prolonged incubation period and is readily transmissible to laboratory rodents. The disease is characterized by progressive neurologic dysfunction caused by degeneration of the C.N.S. Neuropathologic changes include proliferation of glial cells, vacuolation of neurons and depositions of extracellular amyloid in the form of plaques.

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Section: Prion Rods and Filaments Are Amyloidmentioning

confidence: 97%