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. 1995;19(3):231–252. doi: 10.1007/BF01839302

Reflections on scrapie and related disorders, with consideration of the possibility of a viral aetiology

C Darcel 1
PMCID: PMC7088560  PMID: 7571397

Abstract

The transmissible spongiform encephalopathies of domesticated animals, scrapie in-sheep and bovine spongiform encephalopathy (BSE), and transmissible mink encephalopathy are more than a scientific curiosity; under certain circumstances their impact on commercial activities can be calamitous. Knowledge of their causation and pathogenesis is still rudimentary, but many consider than an unconventional agent, the prion (a brain protein, PrP), that is not associated with nucleic acid is involved in both. Others believe that conventional viruses, which replicate by virtue of their nucleic acid-defined genes, are involved in the causation and progression of the encephalopathies but that technical problems have prevented their identification. Others postulate even more exotic causative agents. While this paper will particularly address the possibility of a viral aetiology for these diseases, it is also emphasized that our knowledge of the state of the immune system in animals with encephalopathy needs broadening. There are remarkable gaps in our knowledge of the histopathology of these diseases, particularly the nature of the characteristic vacuoles. Much further work is needed on the biochemical changes in the brain and the serum, particularly of the latter as it could lead to an additional means of recognizing clinical cases without waiting for the animal to die with subsequent examination of the brain for characteristic lesions and the presence of protease-K-resistant PrP.

Keywords: aetiology, Creutzfeldt-Jakob, genes, prion, scrapie, spongiform encephalopathies, transmissible mink encephalopathy, virus

Abbreviations

AI

artificial insemination

BSE

bovine spongiform encephalopathy

CJD

Creutzfeldt-Jakob disease

ET

embryo transfer

GSSD

Gerstmann-Sträussler-Scheinker disease

HDV

hepatitis delta virus

MCF

mink cell focus

PK

proteinase K

PrP

prion protein

PrPSc

scrapie prion protein

PrP-C

the proteinase-K sensitive homologue in normal brain

SAF

scrapie-associated fibrils

TME

transmissible mink encephalopathy

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