Bovine spongiform encephalopathy and food safety
Rosalind Dalefield and Scott CrerarFood Standards Australia New Zealand
PO Box 7186, Canberra BC
ACT 2610, Australia
Tel: +61 2 6271 2222
Fax: +61 2 6271 2278
Email: scott.crerar@foodstandards.gov.au
Microbiology Australia 34(2) 86-89 https://doi.org/10.1071/MA13030
Published: 13 May 2013
Abstract
Bovine spongiform encephalopathy (BSE) is a fatal disease of cattle, caused by infective proteins known as prions. A prion (PrPSc) is a mis-folded isoform of the glycoprotein PrPC, which is highly expressed in the nervous system. Prions replicate by coercing PrPC to refold into PrPSc. The BSE epidemic was propagated by rendering dead cattle to produce meal which was then included in cattle feed. Consumption of BSE PrPSc from contaminated beef resulted in over 200 human cases of variant Creutzfeldt-Jakob (vCJD) disease, which is invariably fatal. There were rare cases of person-to-person vCJD transmission by blood transfusion. Variant CJD is now very rare, due to adoption of measures that prevent the feeding of ruminant protein to ruminants and the contamination of beef with the tissues that harbour PrPSc. Beef from countries with these control systems are safe for human consumption.
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