Prion diseases are slow degenerative brain diseases that occur in people and various other mammals. No vaccines or treatments are available, and these diseases are almost always fatal. Scientists have found little evidence of a protective immune response to prion infections. Further, microglia—brain cells usually involved in the first level of host defense against infections of the brain—have been thought to worsen these diseases by secreting toxic molecules that can damage nerve cells.
Prion News Releases
Chronic wasting disease (CWD) did not cross the species barrier to infect cynomolgus macaque monkeys during a lengthy investigation by National Institutes of Health scientists exploring risks to humans.
Familial human prion diseases are passed within families and are associated with 34 known prion protein mutations. To determine whether three of the unstudied mutations are transmissible, scientists from the National Institute of Allergy and Infectious Diseases, part of the National Institutes of Health, exposed research mice to brain samples from three people who died from a familial prion disease. After observing the mice for about two years, they found two of the mutations, Y226X and G131V, are transmissible.
National Institutes of Health scientists developing a rapid, practical test for the early diagnosis of prion diseases have modified the assay to offer the possibility of improving early diagnosis of Parkinson’s disease and dementia with Lewy bodies. The group, led by NIH’s National Institute of Allergy and Infectious Diseases (NIAID), tested 60 cerebral spinal fluid samples, including 12 from people with Parkinson’s disease, 17 from people with dementia with Lewy bodies, and 31 controls, including 16 of whom had Alzheimer’s disease.
National Institutes of Health (NIH) scientists and collaborators at Case Western Reserve University School of Medicine have detected abnormal prion protein in the skin of nearly two dozen people who died from Creutzfeldt-Jakob disease (CJD). The scientists also exposed a dozen healthy mice to skin extracts from two of the CJD patients, and all developed prion disease.
A product that mimics the natural oxidative killing action of human immune cells against bacteria, viruses, and fungi also can inactivate prions and other proteins, some of which are thought to be important in Parkinson’s and Alzheimer’s diseases, according to National Institutes of Health (NIH) researchers. Prions are deadly protein-based pathogens that are extremely difficult to inactivate; recommended decontamination treatments often are dangerous to people or damaging to surfaces, such as those on surgical devices.