Among the topics are the function of prion protein and the family member shadoo, the effect of microglial inflammation in prion disease
, clinical aspects of human prion diseases
, bovine spongiform encephalopathy and scrapie, and chronic wasting disease: the current assessment of transmissibility.
VPSPr was first reported in 2008 and further defined in 2010 (6-8) as a sporadic prion disease
distinct from sCJD.
Fatal familial insomnia (FFI) is a serious and rare prion disease
, which was first reported by Lugaresi et al.
* Whether the case was reported to the National Prion Disease
Pathology Surveillance Center (NPDPSC) and whether any of the tissue should be referred for further special investigations.
In 1996, after the first report of variant CJD (the human prion disease
caused by the agent of bovine spongiform encepathalopathy ["mad cow disease"]) in the United Kingdom (6), the nongovernmental Japanese CJD Surveillance Committee (J-CJDSC), with support from the Japanese Ministry of Health, Labour, and Welfare, conducted a preliminary nationwide mail survey to identify cases of human prion disease
in Japan; since 1999, J-CJDSC has maintained a national CJD registry (7).
EP-QuIC is 80-90% sensitive and 99-100% specific for the diagnosis of 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 team said this could make a significant contribution to lowering the number of animals used in research in line with the principles of the 3Rs--i.e., reduction, replacement, refinement--which are a central tenet of the ethical use of animals in research.
The most widely recognized human prion disease
, or transmissible spongiform encephalopathy is variant Creutzfeldt--Jakob disease (vCJD) .
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.
The mammalian Pr[P.sup.C] is a highly conserved glycoprotein localized in membrane lipid rafts and anchored to the cell surface by glycosylphpsphatidylinositol (GPI) (28, 29) Pr[P.sup.C] is located in many cell types, and is particularly abundant in neurons (30) Under certain conditions Pr[P.sup.C] may undergo conversion into a conformation-ally altered isoform (Pr[P.sup.Sc]) which is widely believed to be the pathogenic agent in prion disease
A healthy, young, fully responsive immune system, therefore, may be necessary to generate the whole spectrum of prion disease
features in rapid progression.