Creutzfeldt-Jakob Disease Press Releases

Prions have been among the most controversial of infectious disease agents. These misshapen proteins have no DNA or RNA, so many researchers have been skeptical of the idea that they alone can be responsible for disease. Now, infectious prions have successfully been created in the laboratory for the first time, providing insight into how these deadly proteins form.

Most people with a rare type of dementia called primary progressive aphasia (PPA) have a specific combination of prion gene variants, a new study shows. The study is the first to link the prion protein gene to this disorder. The researchers also looked at the prion protein gene in people with Alzheimer's disease and amyotrophic lateral sclerosis (ALS or Lou Gehrig's disease) and did not find any association with specific gene variants in those disorders.

A highly sensitive post-mortem test could help scientists more accurately determine if a person died of Creutzfeldt-Jakob disease (CJD), a human neurological disorder caused by the same class of infectious proteins that trigger mad cow disease, according to a new study supported in part by the National Institutes of Health (NIH). The finding opens the possibility that such testing might be refined in the future so it can be used to detect prion disease in living people and animals before the onset of symptoms.

The combination of high temperature and very high pressure in the preparation of processed meats such as hot dogs and salami may effectively reduce the presence of infective prions while retaining the taste, texture, and look of these meats, according to a new study.
Fact Sheet

A protein widely distributed in tissues throughout the body, with the highest concentration in the brain, has been shown to be a specific marker in the spinal fluid of humans and animals infected with transmissible spongiform encephalopathies, scientists say. This discovery paves the way for the development of an improved test for the diagnosis of Creutzfeldt-Jakob disease in humans and encephalopathies in animals. The test could enable precise identification of disease in British cattle presently targeted for slaughter because of suspected infection with bovine spongiform encephalopathy, known as Mad Cow disease.

Scientists at the National Institute of Neurological Disorders and Stroke (NINDS) have linked three outbreaks of Creutzfeldt-Jakob disease (CJD) in Europe and Israel to a genetic mutation found in the outbreaks' victims.

The combination of high temperature and very high pressure in the preparation of processed meats such as hot dogs and salami may effectively reduce the presence of infective prions while retaining the taste, texture, and look of these meats, according to a new study.
Fact Sheet

Stanley B. Prusiner, M.D., a long-time grantee of the National Institutes of Health (NIH), is the recipient of the 1997 Nobel Prize in physiology or medicine for his discovery of an unusual class of infectious particles called prions. Prions are believed to be responsible for a group of diseases that include "mad cow" disease. Prusiner, who is professor of neurology, virology, and biochemistry at the University of California, San Francisco (UCSF), has received more than 56 million dollars in research grant support from NIH during the last three decades.

A protein widely distributed in tissues throughout the body, with the highest concentration in the brain, has been shown to be a specific marker in the spinal fluid of humans and animals infected with transmissible spongiform encephalopathies, scientists say. This discovery paves the way for the development of an improved test for the diagnosis of Creutzfeldt-Jakob disease in humans and encephalopathies in animals. The test could enable precise identification of disease in British cattle presently targeted for slaughter because of suspected infection with bovine spongiform encephalopathy, known as Mad Cow disease.

Scientists at the National Institute of Neurological Disorders and Stroke (NINDS) have linked three outbreaks of Creutzfeldt-Jakob disease (CJD) in Europe and Israel to a genetic mutation found in the outbreaks' victims.