Prion Disease News and Research

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Fibril shape is the basis of prion strains and cross-species prion infection

New research on prions, the infectious proteins behind "mad cow" disease and Creutzfeld-Jakob disease in humans, suggests that the ability of prions in one species to infect other species depends on the shape of the toxic threadlike fibers produced by the prion. Two studies on the topic appear in the 8 April issue of the journal Cell.

7 Apr 2005

Research into how prions act in the brain could hold the key to defeating diseases

Scientists at the University of Southampton are continuing their pioneering research into diseases such as Creutzfeld-Jakob Disease (CJD) and related conditions caused by tiny particles known as prions, thanks to a £300,000 grant from the Medical Research Council.

1 Mar 2005

Diagnosis of prions in patients should utilize novel strategy, conformation-dependent immunoassay

A technique for detecting prions in tissue, developed in recent years by UCSF scientists, is significantly more sensitive than the diagnostic procedures currently used to detect the lethal particles in samples of brain tissue from patients, according to a study performed by a UCSF team.

22 Feb 2005

Turning off cell suicide switch fails to stop prion damage

Researchers knew that prions, the misfolded proteins that cause mad cow disease and other brain disorders, were killing off a class of important brain cells in a transgenic mouse model. But when they found a way to rescue those cells, they were astonished to discover the mice still became sick.

21 Dec 2004

Movies help explain how proteins gather into clusters called amyloid fibrils

To get a better look at how proteins gather into clusters called amyloid fibrils – which are associated with important human diseases such as Alzheimer’s, Parkinson’s and the so-called prion diseases like Mad Cow – researchers at North Carolina State University decided to make movies.

23 Nov 2004

Mad cow disease prions can be removed from blood

New research results released today confirm that infectious variant Creutzfeldt-Jakob Disease (vCJD) prions that cause the human form of "mad cow disease" can be removed from blood.

From Pall Corporation 3 Nov 2004

Amyloid growth can occur independently of oligomers

Improperly folded proteins are normally destroyed. But in a wide range of diseases, including prion (from proteinaceous and infectious) diseases and neurodegenerative diseases like Parkinson disease and Alzheimer disease, amyloid fibrils, or plaques--misshapen proteins that aggregate into characteristic ropelike configurations--accumulate in tissue.

22 Sep 2004

Second case of variant Creutzfeldt-Jakob disease infection that was probably caused by blood transfusion

Research reported in The Lancet this week details the second case of variant Creutzfeldt-Jakob disease (vCJD) infection that was probably caused by blood transfusion. The rogue prion responsible for vCJD was identified at postmortem five years after an elderly person received a blood transfusion from a donor who later developed vCJD.

5 Aug 2004

Scientists have produced a prion protein that can trigger the development of a neurological disorder similar to "mad cow" disease

Scientists have produced a prion protein that can trigger the development of a neurological disorder in mice that is similar to "mad cow" disease, according to a new study supported by the National Institute on Aging, a part of the National Institutes of Health.

2 Aug 2004

DNA testing of one millionth sheep for scrapie susceptibility

Orchid BioSciences has announced that its animal genetic testing facility in the U.K. has completed DNA testing of its one millionth sheep for scrapie susceptibility.

10 Jul 2004

No evidence of CJD associated with the Garden State Racetrack

The Department of Health and Senior Services released a report today on an investigation of a suspected cluster of Creutzfeldt-Jakob Disease (CJD) associated with the Garden State Racetrack.

8 May 2004