Technology Description:  ARS scientists have developed a test for identifying a gene that could cause Bovine Spongiform Encephalopathy (BSE)—also known as mad cow disease.  Mad cow disease is one of four known animal TSEs (transmissible spongiform encephalopathies), a group of diseases caused by abnormal proteins in the nervous system called prions.  This slow-acting, neurodegenerative disease poses a serious threat to the cattle industry worldwide—resulting in substantial economic losses. This disease also poses safety concerns.  Since 1990, more than 170 people worldwide are believed to have contracted the human prion-related disease called, variant Creutzfeldt-Jakob, from consuming BSE-contaminated food.   While BSE-detection technologies are available, this is the first description of a potential pathogenic mutation within the bovine prion protein gene.   Tests based on this discovery will offer the possibility for genetic surveillance of cattle for this pathogenic mutation.  This technology will also allow for identifying animals for disposal and/or removal from breeding, and should help keep the human food and animal feed chain free from bovine products contaminated with BSE.  This invention is the result of a research project to identify TSE control strategies.  ARS has one of the world's most comprehensive research programs investigating TSEs.   Diagnostic companies and government regulatory agencies could use this tool as a test to detect this potentially pathogenic mutation in cattle and prevent BSE of genetic origin in cattle herds.   Reference:  Please refer to S.N. 11/787,784 (Docket #0078.06), filed on April 8, 2007.  Foreign rights are available.   Inventors: Eric M. Nicholson   USDA-ARS-Virus and Prion Diseases of Livestock   Ames, IA   Phone: (515) 663-7443      Fax: (515) 663-7458   Eric.nicholson@ars.usda.gov