Prion News and Research

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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

Statement from U.S. Department of Agriculture on an inconclusive BSE test result

Statement by Andrea Morgan, Associate Deputy Administrator, Animal and Plant Health Inspection Service, U.S. Department of Agriculture on an Inconclusive BSE Test Result.

18 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

Large-scale screening programme of tonsil tissue is the only way of identifying the true incidence of vCJD infection

A study in this week’s issue of THE LANCET describes how two different types of analysis used in conjunction on samples of tonsil tissue is the ‘gold standard’ method for confirming clinical variant CJD

30 Sep 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

Protein that causes juvenile Batten disease also plays a key role in normal cell function

The aberrant protein that causes juvenile Batten disease, a fatal neurodegenerative disorder of childhood, also plays a key role in normal cell function, Duke University Medical Center researchers have found.

16 Sep 2004

John Reid has announced further developments concerning variant Creutzfeldt-Jakob disease

He told MPs in a written ministerial statement that an exercise has begun to notify some recipients of blood products about the results of a risk assessment exercise carried out by the Health Protection Agency.

10 Sep 2004

Chiron acquires a variant Creutzfeldt-Jakob disease research company

Chiron Corporation has announced that it had acquired Prion Solutions Inc., a privately held company based in La Jolla, Calif., focused on research into variant Creutzfeldt-Jakob disease (vCJD) and other prion-related diseases. Financial terms of the acquisition were not disclosed.

1 Sep 2004

The prion appears to function as an evolutionary stepping stone

“This prion,” explains Lindquist, “has a capacity to hide and release genetic information throughout the entire genome that can contribute to new traits in a complex way.”

16 Aug 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

New precautionary measures against the possible risk of transmission of variant Creutzfeldt Jakob Disease

The UK Department of Health (DOH) has said today that recipients of blood transfusions are to be excluded from donating blood in the future as a further precautionary measure against the possible risk of transmission of variant Creutzfeldt Jakob Disease (vCJD), Health Secretary John Reid announced today.

23 Jul 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

300,000 mad French cows since the 1980's

According to new French research reported in le figaro more than 300,000 French cows would have been infected by the agent BSE (Bovine Spongiform Encephalopathy ) since the 1980's.

3 Jul 2004

Study indicates the number of people in the UK who could be incubating vCJD

The Journal of Pathology recently published a paper entitled Prevalence of lymphoreticular prion protein accumulation in UK tissue samples. The study looked at 12,674 stored appendix and tonsil samples, and found that 3 of them showed evidence of the prion protein associated with variant Creutzfeldt-Jakob Disease (vCJD).

25 May 2004

Are we as crazy as mad cows?

When proteins in our body work properly, we can see, smell, consume and digest food, grow muscle and brain cells. But when these infinitely useful biological building blocks fail, the most pernicious diseases arise. Susan Lindquist has scrutinized the complex origami-like shapes of proteins and come to understand how structural mistakes can lead to a frightening class of neurodegenerative disorders, including “Mad Cow Disease.”

20 May 2004

Natural biological process by which yeast cells dismantle amyloid fibers

Amyloid fibers, those clumps of plaque-like proteins that clog up the brains of Alzheimer's patients, have perplexed scientists with their robust structures. In laboratory experiments, they are able to withstand extreme heat and cold and powerful detergents that cripple most other proteins.

20 May 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

New device filters blood supplies on-site reducing the possible transmission of Creutzfeldt-Jakob disease

ProMetic Life Sciences Inc. has announced today that its joint venture with the American Red Cross, Pathogen Removal and Diagnostic Technology Inc. ("PRDT"), has confirmed that treatment of TSE spiked red blood cell concentrate with its Prion Reduction Device results in a significant decrease in associated infectivity.

5 Apr 2004