Understanding human prion diseases.
Grant number: 454546 | Funding period: 2007 - 2009
The prion diseases are a group of transmissible, neurodegenerative disorders affecting both humans and animals. The most common form in humans is sporadic Creutzfeldt-Jakob disease (CJD), although acquired (variant CJD) and inherited (familial CJD) forms also exist. Prion diseases are transmissible by inoculation with, or dietary exposure to, infected tissues. The infectious agent, referred to as a prion , has not been conclusively identified. However, a major component of purified prions is an abnormal disease associated form of the host prion protein. Differences in the duration of illness and pathology of sporadic CJD suggests that the disease may be caused by different prion strains. The..View full description
Related publications (9)
The Prion Protein Preference of Sporadic Creutzfeldt-Jakob Disease Subtypes
Helen MJ Klemm, Jeremy M Welton, Colin L Masters, Genevieve M Klug, Alison Boyd, Andrew F Hill, Steven J Collins, Victoria A Lawson
Sporadic Creutzfeldt-Jakob disease (CJD) is the most prevalent manifestation of the transmissible spongiform encephalopathies or p..
No evidence for prion protein gene locus multiplication in Creutzfeldt-Jakob disease
Steven J Collins, Maaike Schuur, Alison Boyd, Victoria Lewis, Genevieve M Klug, Amelia McGlade, Andrew van Oosterhout, Guido Breedveld, Ben A Oostra, Colin Masters, Cornelia M Van Duijn
Precedent of causative multiplication of key gene loci exists in familial forms of both Alzheimer's and Parkinson's diseases. Gene..
PrPC-related signal transduction is influenced by copper, membrane integrity and the alpha cleavage site
Cathryn L Haigh, Victoria A Lewis, Laura J Vella, Colin L Masters, Andrew F Hill, Victoria A Lawson, Steven J Collins
The copper-binding, membrane-anchored, cellular prion protein (PrP(C)) has two constitutive cleavage sites producing distinct N- a..
A manganese-superoxide dismutase/catalase mimetic extends survival in a mouse model of human prion disease
Marcus W Brazier, Susan R Doctrow, Colin L Masters, Steven J Collins
Animal models, and human postmortem studies, of prion disease have demonstrated the presence of heightened oxidative stress in the..