New Form of Mad Cow Disease Resembles Human Creuzfeldt-Jakob Disease (CJD)

British cow infected by deadly misshapen proteins called prions which cause "Mad Cow" disease, also known as Bovine Spongiform Encephalopathy (BSE). Cow brain tissue on right is filled with microscopic holes like a sponge, produced by the deaths of brain cells. Photographs courtesy www.mad-cow.org.

Left: is the normal prion protein, PrP-C. Right: is the abnormal, misshapen PrP-SC. These diagrams are from Huang, Z., Prusiner, Stanley B. and Cohen, Fred E. from "Structures of Prion Proteins and Conformational Models for Prion Diseases in Prions," (ed. S.B. Prusiner) Berlin: Springer-Verlag, © 1996. — Top: is the normal prion protein, PrP-C. Bottom: is the abnormal, misshapen PrP-SC. These diagrams are from Huang, Z., Prusiner, Stanley B. and Cohen, Fred E. from "Structures of Prion Proteins and Conformational Models for Prion Diseases in Prions," (ed. S.B. Prusiner) Berlin: Springer-Verlag, © 1996.

February 19, 2004 San Francisco, California - "Mad cow" disease once thought to be confined to England has spread to other countries the past few years, including most recently herds in Canada and Washington State. And this week came another disturbing discovery: Italian researchers have found a second type of deadly prion disease in cattle that closely resembles the prion proteins that sporadically and spontaneously attack and kill humans in what is called Creutzfeldt-Jakob Disease, or CJD. That means controlling what goes into cattle feed won't stop all mad cow disease - and that there are even more deadly prions in the food chain than anyone knew until now.

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