Scrapie, a naturally occurring neurodegenerative disease of sheep
and sometimes goats, is a prototypic disease for the whole group of
the subacute spongiform virus encephalopathies. Kuru was the first
human disease of this type to be discovered in 1957 by Gajdusek and
Zigas, and its discovery opened the whole field in the human
biomedical sciences by the very realization of the fact that
viruses may induce disease months or even decades after infections,
and that these slow virus diseases are more compatible with
classical degenerations of the nervous system than with
inflammatory disorders of the brain. More than a quarter of a
century since discovery of Kuru, and more than half a century
following the first transmission of scrapie, the very nature of the
infectious virus remains unknown. This comprehensive review covers
all aspects of slow unconventional virus infections known today. It
includes numerous historical data, biochemistry and molecular
biology of the prion protein and its gene, the role of genetics and
mutations within PrP gene, spreading and targeting of the virus,
biochemistry and neurochemistry of the alterations of different
neurotransmitter system and neuropathology. More than 1000
references are listed and critically analyzed; the reader can find
references to all experiments and laboratory findings which has
ever been done in this field. Furthermore, the book offers
different view on the basic problems as for example, the nature of
the scrapie agent.
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