Creutzfeldt Jakob Disease News and Research

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Creutzfeldt–Jakob disease or CJD (sometimes incorrectly referred to as mad cow disease) is a degenerative neurological disorder (brain disease) that is incurable and invariably fatal. It is the most common among the types of transmissible spongiform encephalopathy found in humans.

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 mechanism identified to protect the brain from many neurodegenerative conditions

Scientists at Case Western Reserve University School of Medicine have identified a novel mechanism that could be used to protect the brain from damage due to stroke and a variety of neurodegenerative conditions, including sporadic Creutzfeldt-Jakob disease, Alzheimer's disease, and Parkinson's disease.

8 Mar 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

Pioneering ultrasonic device can improve cleaning of medical instruments, reduce risk of infection

Researchers from the University of Southampton have demonstrated how a pioneering ultrasonic device can significantly improve the cleaning of medical instruments and reduce contamination and risk of infection.

16 Sep 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

Shire enters into agreement with Sanquin for CINRYZE

Shire plc announced today it has entered into an agreement with Sanquin Blood Supply, the manufacturer of CINRYZE (C1 esterase inhibitor [human]), providing Shire access to its manufacturing technology and allowing Shire to source additional manufacturers to meet the growing demand for CINRYZE.

25 Aug 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

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

Grass plants can bind, uptake and transport infectious prions

Grass plants can bind, uptake and transport infectious prions, according to researchers at The University of Texas Health Science Center at Houston (UTHealth).

18 May 2015

WHO calls on experts, social media to follow best practices in naming new human infectious diseases

WHO today called on scientists, national authorities and the media to follow best practices in naming new human infectious diseases to minimize unnecessary negative effects on nations, economies and people.

8 May 2015

WHO issues best practices for naming new human infectious diseases

The World Health Organization (WHO) today called on scientists, national authorities and the media to follow best practices in naming new human infectious diseases to minimize unnecessary negative effects on nations, economies and people...

8 May 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

UC Berkeley, UCSF researchers team up to create center for neurodegenerative disease research

Researchers at UC San Francisco and UC Berkeley have teamed up to create an innovative, integrated center for research on neurodegenerative diseases. Supported by a $3 million grant from the Glenn Foundation for Medical Research, the new center aims to pave the way to developing novel treatments for diseases such as Alzheimer's disease and Parkinson's disease by investigating the many ways that proteins can malfunction within cells.

10 Oct 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