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431 —
This similarity has stimulated intense research effort, much of it
aimed at the identification of the causal agents. The relation of
Scrapie to human disease and ageing has recently been reviewed by
FIELD
(1976).
The acceptance of the essential unity of these diseases
(GIBBS
and GAJDUSEK, 1971; GAJDUSEK, 1977) and the
continued
emphasis on their transmission to primates has provoked public
health controls on the disposal of Scrapie-affected and in-contact
sheep in the United States.
The causal agent of Scrapie, which is highly resistant to a wide
range of physicochemical treatments including irradiation, is trans-
missible, filterable and capable of replication. The experimental
transmission to a variety of animal species which develop characte-
ristic disease after varying periods of incubation is the only way in
which the agent can be recognised and quantified.
The literature on the nature of the Scrapie agent has been revie-
wed by
HUNTER
and
MILLSOM
(1977)
and from research to date has
emerged the widely held hypothesis that the informational molecule
is likely to be a small membrane bound nucleic acid. Such a small
DNA molecule has been isolated from mouse and hamster Scrapie
brain and the main objective of further work is to determine whe-
ther Scrapie-specific information is coded by this molecule (ARC
Report,
1977).
The clinical syndrome of Scrapie can vary in diffe-
rent breeds and species reflecting not only genetic differences bet-
ween the hosts but also strain differences of the agent. The incuba-
tion period and the rate of progression of clinical symptoms is rela-
ted to the strain used. Moreover, experimental evidence in mice sup-
ports the view that different strains of the Scrapie agent can com-
pete for a limited number of multiplication sites and the inoculation
of « slow » agents can prevent « faster » agents injected later from
producing disease.
The utilisation of genetic selection for resistance in sheep enabled
two lines differing in susceptibility by
90%
to be established in a
group of Cheviot sheep (DICKINSON et al.,
1968);
a similarly resis-
tant line of Herdwick sheep was also established (NUSSBAUM et al.,
1975).
However, the occurrence of an outbreak of natural Scrapie
recently in sheep in susceptible lines of both breeds unrelated to a
component of the SSBP/1 challenge strain emphasises the duplicity
of strains of the agent. Nevertheless, HOARE et al.
(1977)
have esta-
blished a nucleus breeding flock of Swaledale sheep comprising
those surviving a Scrapie challenge and these in turn produced off-