Prion News and Research

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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

Design of artificial prion may aid fight against mad cow disease

The culprit behind mad cow disease, a.k.a. bovine spongiform encephalopathy, is the most infamous mammalian form of prions. Prions are misfolded proteins that are capable of growing, replicating, and being passed on to daughter cells, that is, they are by themselves heritable. Beyond their disease manifestation, prions also occur naturally in some organisms (such as yeast) and may play important roles in their growth and development. Now, Osherovich and colleagues have identified the amino acid sequences that allow prions to aggregate and replicate, and thereby pass through generations of cells - and prove this by designing an artificial yeast prion that does not exist in nature.

23 Mar 2004