Prion Disease News and Research

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Researchers clarify beneficial function of brain protein linked to mad cow disease

Scientists have clarified details in understanding the beneficial function of a type of protein normally associated with prion diseases of the brain, such as bovine spongiform encephalopathy (commonly known as mad cow disease) and its human counterpart, variant Creutzfeldt-Jakob disease.

8 Aug 2016

Researchers develop new screening system to measure protein aggregates

Researchers from the Massachusetts Institute of Technology and Boston University have developed a system capable of quickly screening millions of yeast cells to measure protein aggregates.

15 Jul 2016

Study finds link between copper exposure and prion protein neurotoxicity

Iowa State University researchers have described with single-molecule precision how copper ions cause prion proteins to misfold and seed the misfolding and clumping of nearby prion proteins.

4 Jul 2016

UTHealth researchers receive $11 million grant to study prion diseases

Led by Claudio Soto, Ph.D., researchers from McGovern Medical School at The University of Texas Health Science Center at Houston have been awarded $11 million from the National Institute of Allergy and Infectious Diseases to study the pathogenesis, transmission and detection of prion diseases - such as chronic wasting disease in deer - that can potentially spread to humans.

30 Jun 2016

Advanced assay offers better sensitivity for detecting a prion disease affecting elk

Transmissible spongiform encephalopathies (TSEs), or prion diseases, are a family of rare progressive, neurodegenerative illnesses that affect both humans and animals. TSE surveillance is important for public health and food safety because TSEs have the potential of crossing from animals to humans, as seen with the spread of mad cow disease, or bovine spongiform encephalopathy (BSE). A study in The Journal of Molecular Diagnostics describes an advanced assay that offers better sensitivity than currently available tests for detecting a prion disease affecting elk.

11 Apr 2016

Novel quantitative method may reduce or eliminate need for invasive biopsies

Scientists have identified a quantitative method to measure changes in biomarkers, which may reduce or eliminate the need for invasive biopsies. The method, described in the February 2016 issue of The FASEB Journal uses a novel chimera design of DNA and small DNA with a companion contrast agent to allow antibodies to cross cellular membranes.

8 Feb 2016

Alpha-synuclein protein involved in Parkinson's disease pathology does not behave like a 'prion'

In Parkinson's disease, the protein "alpha-synuclein" aggregates within neurons of patients and appears to propagate across interconnected areas of the brain. How this happens remains largely unknown.

6 Jan 2016

Scientists propose that Alzheimer's disease should be treated separately

Deciphering the mechanism that underlies the development of Alzheimer's disease in certain families but not in others, researchers at the Hebrew University of Jerusalem's Faculty of Medicine have proposed that the malady is actually a collection of diseases that probably should be treated with a variety of different approaches.

4 Nov 2015

Signs of Alzheimer’s found in growth hormone patients

People may be able to contract Alzheimer’s disease during certain medical procedures in the same way that Creutzfeldt-Jakob disease (CJD) is transmitted, suggest researchers from University College London.

10 Sep 2015

Newly discovered prion causes Multiple System Atrophy

Multiple System Atrophy (MSA), a neurodegenerative disorder with similarities to Parkinson's disease, is caused by a newly discovered type of prion, akin to the misfolded proteins involved in incurable progressive brain diseases such Creutzfeldt-Jakob Disease (CJD), according to two new research papers led by scientists at UC San Francisco.

1 Sep 2015

Prion protein could play a role in depression

The discovery of antidepressant drugs in the 1950s led to the first biochemical hypothesis of depression, known as the monoamine hypothesis. This hypothesis proposes that an imbalance of certain brain chemicals is the key cause of depression.

14 Aug 2015

New study reveals how infectious proteins spread from gut to the brain

Diagnosis of deadly brain conditions could be helped by new research that shows how infectious proteins that cause the disease spread.

5 Aug 2015

New monoclonal antibodies may provide blueprint for effective Alzheimer's disease treatments

Scientists at NYU Langone Medical Center's Center for Cognitive Neurology have evidence that monoclonal antibodies they developed may provide the blueprint for effective treatments for Alzheimer's disease and other neurodegenerative diseases, such as Parkinson's disease.

21 Jul 2015

Prion variant offers “dramatic” protection against prion disease

A naturally occurring variant of the human prion protein is not only resistant to conversion into an abnormal form but also prevents the conversion of wild-type proteins, shows a study in Nature.

12 Jun 2015

Study identifies small loop in human prion protein that resists chronic wasting disease

Chronic wasting disease (CWD) — an infectious disease caused by prions — affects North American elk and deer, but has not been observed in humans. Using a mouse model that expresses an altered form of the normal human prion protein, researchers at University of California, San Diego School of Medicine have determined why the human proteins aren't corrupted when exposed to the elk prions.

24 Feb 2015

TSRI scientists discover novel mechanism involved in Alzheimer's, Parkinson's and ALS

Scientists from the Florida campus of The Scripps Research Institute have for the first time discovered a killing mechanism that could underpin a range of the most intractable neurodegenerative diseases such as Alzheimer's, Parkinson's and ALS.

16 Feb 2015

'Mad Cow' discovery points to possible neuron killing mechanism behind alzheimer’s and parkinson’s diseases

The new study, published recently in the journal Brain, revealed the mechanism of toxicity of a misfolded form of the protein that underlies prion diseases, such as bovine spongiform encephalopathy (“mad cow disease”) and its human equivalent, Creutzfeldt-Jakob disease.

13 Feb 2015

Scrapie pathogens can convert human prion protein to a pathological state

INRA scientists have shown for the first time that the pathogens responsible for scrapie in small ruminants (prions) have the potential to convert the human prion protein from a healthy state to a pathological state. In mice models reproducing the human species barrier, this prion induces a disease similar to Creutzfeldt-Jakob disease.

24 Dec 2014

First successful vaccination of deer against chronic wasting disease

Researchers at NYU Langone Medical Center and elsewhere say that a vaccination they have developed to fight a brain-based, wasting syndrome among deer and other animals may hold promise on two additional fronts: Protecting U.S. livestock from contracting the disease, and preventing similar brain infections in humans.

23 Dec 2014

Infectious prion protein is marker for variant Creutzfeldt-Jakob disease

The misfolded and infectious prion protein that is a marker for variant Creutzfeldt-Jakob disease - linked to the consumption of infected cattle meat - has been detected in the urine of patients with the disease by researchers at The University of Texas Health Science Center at Houston (UTHealth) Medical School.

8 Aug 2014