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Is there more to prions than BSE?
Max Pruetzel-Thomas, Reporter, Central & Eastern Europe

Scientist's impression of the prion protein. UK researchers believe BSE is not the only brain disease linked to prion proteins
Photo: CABM Structural Bioinformatics Laboratory/ Rutger University, New Jersey

Prion proteins may be associated not only with bovine spongiform encephalopathy (BSE), but also with other brain diseases of cattle.

Scientists in the UK have found changes in the production and accumulation of prion proteins in the brains of cattle with idiopathic brainstem neuronal chromatolysis (IBNC), a rare neurodegenerative disorder.

A team of researchers from the Veterinary Laboratories Agency made the discovery after testing the brains of 15 cattle with IBNC, a disease first characterized in 1988 in cattle suspected of having BSE. Although the two diseases have some similarities, the brains of cattle with IBNC do not have the lesions typical of those with BSE.

Laboratory tests suggest that the misfolded form of prion protein which causes BSE is not present in cattle with IBNC. Commercial BSE testing kits also failed to detect this form of prion protein. However, tests did reveal increased levels of normal prions.

"We've shown for the first time that prion protein is somehow involved in IBNC," said Dr Martin Jeffrey, who led the study. "In this disease there is an association with abnormally high levels of prion protein in the brain, but clearly this PrP is in a different form to that involved in BSE and CJD. This may have implications for the diagnosis and recognition of typical forms of BSE as well as the related diseases in sheep, deer and man."

The findings are published in the open access journal BMC Veterinary Research.

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