Why do prions cause disease

Privacy Policy. Create an account. Password recovery. All Trends for Trends for Identifying the Next Frontier in Vaccine Manufacturing. Educating Next-Generation Bioprocess Engineers. Biopharma Demands Create an Innovation Ecosystem. Top 10 Spatial Biology Companies. Top 10 Synthetic Biology Companies. Mutations that cause prion disease are clustered within or adjacent to key structural elements in the protein, so it is easy to imagine that mutations destabilize the structure of pN and cause it to reconfigure into pD.

We do not yet know why the pD structure of a prion would result in neurodegeneration, but we do know that prion protein accumulates in brain tissue. One part of the prion protein can cause apoptosis, or programmed cell death; perhaps this mechanism explains the pattern of the disease. Recently the general public has become interested in them as well because of the epidemic of BSE, more dramatically known as mad cow disease.

Hundreds of thousands of infected animals have been eaten by Europeans and particularly the British over the past 10 years. The latest research suggests that the infected meat may pose a threat to human health, but the significance of that threat may not become apparent for years. Although it is generally considered a British problem, BSE is almost certainly a natural disease of cattle, so it is undoubtedly found in other countries as well.

The normal incidence of BSE is vanishingly small, however. The U. Already a subscriber? Sign in. Thanks for reading Scientific American. Create your free account or Sign in to continue. See Subscription Options. Discover World-Changing Science. She responds: "'Prion' is a term first used to describe the mysterious infectious agent responsible for several neurodegenerative diseases found in mammals, including Creutzfeldt-Jakob disease CJD in humans.

Get smart. Sign up for our email newsletter. Sign Up. Support science journalism. Knowledge awaits. See Subscription Options Already a subscriber? Create Account See Subscription Options. Continue reading with a Scientific American subscription. They are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss, and a failure to induce inflammatory response.

The causative agents of TSEs are believed to be prions. The functions of these normal prion proteins are still not completely understood. The abnormal folding of the prion proteins leads to brain damage and the characteristic signs and symptoms of the disease. Prion diseases are usually rapidly progressive and always fatal.

Listed below are the prion diseases identified to date. CDC does not currently offer information on every prion disease listed below. Classic CJD is a human prion disease. It is a neurodegenerative disorder with characteristic clinical and diagnostic features.