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Study sheds light on why normal prion proteins may experience age-related mutations in inherited diseases

A new study sheds light on why normal prion proteins may experience age-related mutations in inherited diseases. Researchers at the Case Western Reserve University School of Medicine studied a previously discovered mutation in the prion protein in members of an extended family in Indiana who inherited the prion disease Gerstmann-Straussler-Sheinker (GSS).

25 Apr 2005

Strongest evidence yet that infectious misformed proteins cause mad cow disease

Researchers at the University of Texas Medical Branch at Galveston (UTMB) have produced the strongest proof yet that the mysterious and devastating brain diseases known as "transmissible spongiform encephalopathies" (TSEs) are transmitted by an infectious agent composed only of a malformed protein, and not a virus.

21 Apr 2005

Researchers and drug giant team up to find cure for CJD

The Medical Research Council's Prion Unit, based at University College London, a leading British research group, is joining forces with drugs giant GlaxoSmithKline (GSK) to develop a cure for the disease Creutzfeldt-Jakob Disease (CJD).

12 Apr 2005

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

Prions, previously thought to accumulate mainly in the brain can also infect other organs

Prions, infectious proteins associated with bovine spongiform encephalopathy (BSE) or Mad Cow Disease, were previously thought to accumulate mainly in the brain, but Yale and University of Zurich researchers report in Science that other organs can also become infected.

7 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

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