Transmissible Spongiform Encephalopathies

According to the World Health Organization, transmissible spongiform encephalopathies (TSEs) are "a family of diseases of humans and animals characterized by spongy degeneration of the brain with severe and fatal neurological signs and symptoms."[1] They are "a group of rare degenerative brain disorders characterized by tiny holes that give the brain a 'spongy' appearance. These holes can be seen when brain tissue is viewed under a microscope."[2]

Diseases in the TSE family are believed to be caused by prions, abnormal versions of proteins present in a wide range of mammalian species. The TSE disease family includes[3]:

• Scrapie (in sheep and goats)
• Bovine Spongiform Encephalopathy (in cattle, but transferable to other animals)
• Transmissible Mink Encephalopathy (in mink)
• Chronic Wasting Disease (in mule deer and elk)
• Feline Spongiform Encephalopathy (in cats)
• Exotic Ungulate Encephalopathy (in greater kudu, nyala and oryx)
• Kuru (human disease caused by ritualistic cannibalism formerly practiced by the Fore people in Papua New Guinea)
• Iatrogenic Creutzfeldt-Jakob Disease (human disease caused by prion-contaminated medical equipment, human growth hormone, tissue grafts, etc)
• Sporadic Creutzfeldt-Jakob Disease (human disease believed to be caused by spontaneous, non-inherited mutations in the prion gene or spontaneous conversion of prion protein to the abnormal, disease-causing configuration)
• Variant Creutzfeldt-Jakob Disease (human disease believed to be caused by ingesting prion-contaminated animal products)
• Familial Creutzfeldt-Jakob Disease (human disease caused by inherited mutations in the prion gene)
• Gerstmann-Sträussler-Scheinker Disease (human disease caused by inherited mutations in the prion gene)
• Fatal Familial Insomnia (human disease caused by a distinct, inherited prion gene mutation, D178N and M129)

SourceWatch Resources

• Mad cow disease - contains list of links to TSE/mad cow-related entries

External links

• National Institute of Neurological Disorders and Stroke (U.S.), "NINDS Transmissible Spongiform Encephalopathies Information Page," December 2001.
• TSE News and Headlines, from Microbes.info