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.

Unraveling the role of liquid-liquid phase separation in neurodegenerative diseases

Liquid-liquid phase separation is not a precursor to formation of amyloid fibrils, a pathological hallmark of Parkinson's disease, shows study.

15 Jul 2024

Mammal-specific domain of the prion protein may hold clues to neurodegenerative diseases

A study from Bochum describes a mammal-specific domain of the prion protein and offers new approaches for research into neurodegenerative diseases.

23 May 2024

Study confirms strong barrier preventing chronic wasting disease transmission to humans

A new study of prion diseases, using a human cerebral organoid model, suggests there is a substantial species barrier preventing transmission of chronic wasting disease (CWD) from cervids-;deer, elk and moose-;to people.

17 May 2024

Repurposing human-approved drugs for prion disease treatment

Prions are the abnormal, pathogenic agents that are transmissible and are able to induce abnormal folding of specific normal cellular proteins.

16 May 2024

Possibility of wildlife-to-human crossover heightens concern about chronic wasting disease

Each fall, millions of hunters across North America make their way into forests and grasslands to kill deer. Over the winter, people chow down on the venison steaks, sausage, and burgers made from the animals.

2 Feb 2024

Novel learning-based framework for predicting Alzheimer’s disease progression

About 55 million people worldwide are living with dementia, according to the World Health Organization. The most common form is Alzheimer's disease, an incurable condition that causes brain function to deteriorate.

27 Jan 2024

New insights into the interplay between prions and TDP-43 in neurodegenerative pathology

Neurodegenerative diseases are characterized by the deposition of clumped proteins in the brain and progressive neuronal cell death.

4 Dec 2023

Novel prion strain causes chronic wasting disease in a Finland moose

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19 Jan 2023

Brain changes can be studied in very early stages of neurodegenerative diseases

Many changes take place in the brain long before symptoms appear. This has been shown in mice in two studies of prion diseases in which the brain gradually deteriorates.

10 Oct 2022

Decline in specific protein levels linked to Alzheimer’s disease, not buildup of amyloid plaques

New research from the University of Cincinnati bolsters a hypothesis that Alzheimer's disease is caused by a decline in levels of a specific protein, contrary to a prevailing theory that has been recently called into question.

5 Oct 2022

NPDPSC at Case Western Reserve University to receive CDC funding for better understanding of prion diseases

The National Prion Disease Pathology Surveillance Center at Case Western Reserve University will receive up to $20 million in funding as part of a grant renewal from the Centers for Disease Control and Prevention (CDC) to better understand how and why prion diseases––degenerative brain conditions found in both humans and animals-;develop and spread.

20 Sep 2022

Researchers uncover the structure of protein fibrils linked to hereditary form of human prion disease

In a new study, researchers from the Case Western Reserve University School of Medicine have identified the structure of protein fibrils linked to a hereditary form of human prion disease.

12 Sep 2022

Prion imaging could lead to better understanding of what causes fatal neurological disorders

Prion diseases are incurable, deadly neurological disorders that can affect both humans and animals–including Creutzfeldt-Jakob Disease (CJD) in people, Bovine Spongiform Encephalopathy (also known as Mad Cow Disease) and Chronic Wasting Disease found in deer.

13 Jul 2022

New technologies for early clinical diagnosis of human prion diseases

Early and accurate diagnosis of human prion diseases is a long-standing difficulty. Currently, the definitive diagnosis of human prion diseases relies on pathognomonic histological features or PrPSc detection of patients' brain tissue biopsy or autopsy samples, which is not feasible in most cases.

11 Jan 2022

New study shines light on how brain cells die in prion diseases

Prion diseases, such as Creutzfeldt-Jakob Disease (CJD), are fast-moving, fatal dementia syndromes associated with the formation of aggregates of the prion protein, PrP.

22 Dec 2021

Research may provide clues to how viral infections contribute to neurodegeneration

Some viral diseases could possibly contribute to neurodegeneration. DZNE researchers report this in the scientific journal "Nature Communications".

19 Oct 2021

Human cerebral organoid model to screen drugs for treatment of Creutzfeldt-Jakob disease

Approximately two years after establishing a human cerebral organoid system to study Creutzfeldt-Jakob disease (CJD), National Institutes of Health researchers have further developed the model to screen drugs for potential CJD treatment.

9 Mar 2021

Study provides new information about the structure of prion proteins

Prion diseases are a group of rapidly progressive, fatal and infectious neurodegenerative disorders affecting both humans and animals: Bovine Spongiform Encephalopathy (BSE) or 'mad cow' disease is one of the most famous since in 1996 scientists found that the agent responsible for the disease in cows, is the same agent responsible for the so-called variant Creutzfeldt-Jakob Disease (vCJD), a disease affecting humans.

20 Feb 2021

Study finds locations of early prion protein accumulation in the eye

The earliest eye damage from prion disease takes place in the cone photoreceptor cells, specifically in the cilia and the ribbon synapses, according to a new study of prion protein accumulation in the eye by National Institutes of Health scientists.

29 Jan 2021

Fatal chain reaction in Alzheimer's disease starts much earlier than commonly assumed

Alzheimer's disease develops over decades. It begins with a fatal chain reaction in which masses of misfolded beta-amyloid proteins are produced that in the end literally flood the brain.

18 Nov 2020