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In early 1996,
the CJD surveillance unit in Edinburgh reported that they had identified 14 cases of a new
variant form of CJD (vCJD) most of whom had a history of close contact with, or
consumption of, BSE-infected meat [30]. By
November 2000 the number of cases in the UK had risen to over 80, and there were a few in
France. Is this the beginning of a large epidemic of vCJD, with hundreds, perhaps
thousands, of cases? It is an appalling thought! The agent responsible for TSEs is
called a prion, or infectious proteinaceous particle, so named by its discoverer, Stanley
Prusiner, in 1982; the discovery led to Prusiner's Nobel Prize award in 1997 [31]. Prions are weird. They are at the border
between living and non-living, incapable of replicating, but they transform host protein
(nerve tissue) into pathological structures that undergo spongiform degeneration. The mode
of transmission appears to be by ingestion. |