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K-State professor's USDA research shows mad cow disease also caused by genetic mutation
Date:9/11/2008

MANHATTAN, KAN. -- New findings about the causes of mad cow disease show that sometimes it may be genetic.

"We now know it's also in the genes of cattle," said Juergen A. Richt, Regents Distinguished Professor of Diagnostic Medicine and Pathobiology at Kansas State University's College of Veterinary Medicine.

Until several years ago, Richt said, it was thought that the cattle prion disease bovine spongiform encephalopathy -- also called BSE or mad cow disease -- was a foodborne disease. But his team's new findings suggest that mad cow disease also is caused by a genetic mutation within a gene called Prion Protein Gene. Prion proteins are proteins expressed abundantly in the brain and immune cells of mammals.

The research shows, for the first time, that a 10-year-old cow from Alabama with an atypical form of bovine spongiform encephalopathy had the same type of prion protein gene mutation as found in human patients with the genetic form of Creutzfeldt-Jakob disease, also called genetic CJD for short. Besides having a genetic origin, other human forms of prion diseases can be sporadic, as in sporadic CJD, as well as foodborne. That is, they are contracted when people eat products contaminated with mad cow disease. This form of Creutzfeldt-Jakob disease is called variant CJD.

"Our findings that there is a genetic component to BSE are significant because they tell you we can have this disease everywhere in the world, even in so-called BSE-free countries," Richt said.

An article by Richt and colleague Mark Hall of the National Veterinary Services Laboratories in Ames, Iowa, was published online in the journal PLoS Pathogens. Richt conducted the research while working at the National Animal Disease Center operated in Ames, Iowa, by the U.S. Department of Agriculture's Agricultural Research Service.

Richt said that prion diseases including mad cow disease are referred to as "slow diseases."

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Contact: Juergen A. Richt
jricht@vet.k-state.edu
785-532-2793
Kansas State University
Source:Eurekalert  

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