Submitted to: Meeting Abstract
Publication Type: Abstract Only
Publication Acceptance Date: 12/12/1996
Publication Date: N/A
Technical Abstract: Bovine spongiform encephalopathy (BSE) is a member of a group of diseases called the transmissible spongiform encephalopathies (TSE's) or prion diseases. The outbreak of BSE, which began in 1985, has caused severe financial loss to the cattle and related industries of Great Britain partly because of great concern that the disease would spread to humans. A Avariant form of CJD (v-CJD), described in 1996, that affects younger peopl than conventional CJD has dramatically heightened this concern. However, there is no definitive evidence of natural transmission of animal prion diseases to humans but many, including human prion diseases can be experimentally transmitted to other species of animals. Because of our long inconsequential association with scrapie, including negative results of multiple comparative studies between groups of exposed and unexposed people, the risk from scrapie is considered nil. That differs from our association with BSE, a relatively new disease that has been recognized fo little longer than the minimal incubation of most prion disease of humans, thus the association is uncertain. Two factors determine the risk of consumers and farmers in the United States to BSE - these are exposure and susceptibility. BSE has not been recognized in the United States but there are suspicions that it occurs rarely because of spontaneous mutations in the prion gene. The susceptibility of humans to BSE is un- known. The human prion gene differs from the cattle gene at about 30 sites. But, whether this is sufficient for protection remains a mystery. Several natural and experimental studies are ongoing that will eventually reveal the facts about transmissibility of BSE to humans.