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Revising the meaning of 'prion'
(Whitehead Institute for Biomedical Research) Prions are infamous for causing Creutzfeld-Jakob disease, fatal familial insomnia, and bovine spongiform encephalopathy, commonly known as mad cow's disease. Yet, it's not likely that's all they're good for. Using an unbiased screen in yeast, a team of Whitehead Institute and Stanford University scientists have identified dozens of prion-like proteins that could change the defining characteristics of these unusual proteins. (Source: EurekAlert! - Infectious and Emerging Diseases)
Source: EurekAlert! - Infectious and Emerging Diseases - October 4, 2016 Category: Infectious Diseases Source Type: news

Nine News' Hayley Webb and her brother inherited Fatal Familial Insomnia
Queensland siblings Hayley and Lachlan Webb have inherited an extremely rare and fatal brain disease called Fatal Familial Insomnia, which leads to rapid mental and physical deterioration. (Source: the Mail online | Health)
Source: the Mail online | Health - May 2, 2016 Category: Consumer Health News Source Type: news

Can't Sleep? Learn about a Rare Type of Insomnia that Can Cause Dementia
For those who struggle with insomnia, lying awake at night can be extremely frustrating. But for a very few people in the world, it can be deadly. Fatal familial insomnia is a rare neurological disorder that causes sleep problems, difficulties in movement, personality changes and dementia. It's a progressive disease, meaning that it gets worse over time and eventually leads to death. Like Creutzfeldt-Jakob disease (the more well-known and often misnamed "mad cow disease"), fatal familial insomnia is a hereditary prion disease. Fortunately, it's extremely rare, so the next time you're unable to sleep, this is on...
Source: About Alzheimers Disease - October 27, 2013 Category: Consumer Health News Source Type: news

New models advance the study of deadly human prion diseases
(Whitehead Institute for Biomedical Research) By directly altering the gene coding for the prion protein, Whitehead Institute researchers have created mouse models of two neurodegenerative prion diseases, each of which manifests in different regions of the brain. These new models for fatal familial insomnia and Creutzfeldt-Jakob disease accurately reflect the distinct patterns of destruction caused by the these diseases in humans. Remarkably, as different as each disease is, they both spontaneously generate infectious prions. (Source: EurekAlert! - Medicine and Health)
Source: EurekAlert! - Medicine and Health - August 19, 2013 Category: Global & Universal Source Type: news

Important step toward pharmacologic development of treatment for prion diseases
A team of researchers from Case Western Reserve University School of Medicine have identified a mechanism that can prevent the normal prion protein from changing its molecular shape into the abnormal form responsible for neurodegenerative diseases. This finding, published in the journal Cell Reports, offers new hope in the battle against a foe that until now has always proved fatal. Prion diseases include Creuzfeldt-Jakob disease and fatal familial insomnia. Unlike other transmissible diseases, the infectious agent is not a virus or bacteria, but an abnormally shaped prion protein... (Source: Health News from Medical News Today)
Source: Health News from Medical News Today - July 23, 2013 Category: Consumer Health News Tags: CJD / vCJD / Mad Cow Disease Source Type: news