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
The research also demonstrates the potential of human stem-cell derived astrocytes to notably reduce, and in many instances, replace animal studies of human prion disease
The topics include protein quality control in health and disease, the HET-S/s prion motif in the control of programmed cell death, cross-beta polymerization of low complexity sequence domains, neurodegenerative disease transmission and transgenesis in mice, potential pathways of abnormal tau and alpha-synuclein dissemination in sporadic Alzheimer's and Parkinson's diseases, and aggregation and prion-like properties of misfolded tumor suppressors: whether cancer is a prion disease
We observed no clinical signs of prion disease
People who eat people with a type of prion disease
, a category of illness that also includes Creutzfeldt-Jakob disease in humans and Mad Cow Disease in animals, tend to have brains that look like sponges and neurological damage that takes away control over the body.
Brain damage was prevented in mice with prion disease
, and animals with frontotemporal dementia (FTD) reclaimed their memory.
This is a prion disease
found in deer and elk that is similar to other prion diseases
such as Mad Cow in cattle (that humans can get), and Creutzfeldt-Jakob Disease (found only in humans).
In 2011, the Boston-area law school graduate learned she carries the same genetic mutation that caused her mother's death from a rare brain-wasting prion disease
The Vesiculovirus Piry infection generates human disease characterized by rapid onset, high fever, headache, chills, photophobia, myalgia, dizziness, and weakness  and, in adult mice, a nonlethal CNS infection and injury to the limbic system including the hippocampus , a target region of the degenerative process induced by prion disease
in mice .
The definite diagnosis was confirmed by the National Prion Disease
Pathology Surveillance Center (NPDPSC), in Cleveland, Ohio, USA.
A prion disease
called Bovine Spongiform Encephalopathy (BSE) is endemic to cows.
The topics range from the suspected neuroprotective role of the non-prion PrPC protein in the brain to the inflammation caused by prion aggregates, molecular mechanisms used by prions, attempts to protect against prion transmission, attempts to develop antibody-based vaccines and therapies for prion diseases
, and the clinical aspects and classification of human prion disease