Prion disease

1 2 How brain cells die in prion diseases Date: December 22, 2021 Source: Scripps Research Institute Summary: 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. How these aggregates form within and kill brain cells has never been fully understood, but a new study suggests that the aggregates kill neurons by damaging their axons, the narrow nerve fibers through which they send signals to other neurons. Share: 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. How these aggregates form within and kill brain cells has never been fully understood, but a new study from scientists at Scripps Research suggests that the aggregates kill neurons by damaging their axons, the narrow nerve fibers through which they send signals to other neurons. The accumulation of protein aggregates in axons, along with axonal swellings and other signs of dysfunction, are also early features of other neurodegenerative disorders including Alzheimer's and Parkinson's diseases. The discovery of how these prion aggregates form in axons and how to inhibit them, reported in Science Advances, may ultimately have a significance that goes far beyond prion diseases. "We're hopeful that these findings will lead to a better understanding of prion and other neurodegenerative disea...

nervous system disease: Prions Diseases caused by prions that affect humans include: Gerstmann-Sträussler-Scheinker disease, chronic wasting disease of slow infections. The pathogenic agent of these diseases does have certain viral attributes, such as extremely small size and strain variation, but other properties are

• • Prion diseases articles from across Nature Portfolio Definition Prion diseases are a group of progressive neurodegenerative diseases that are caused by misfolded proteins, referred to as prions. During post-translational modification of proteins, prions act as a folding template, converting proteins into infectious prion form. The best-known human prion disease is Creutzfeld–Jakob disease. Cultured brain slices from mice spontaneously upregulate senescence-associated genes over time and reproduce the transcriptional characteristics of aged brains; prions accelerate brain aging in brain slice cultures, animal models and human patients. • Yingjun Liu • , Assunta Senatore • & Adriano Aguzzi A new study shows that interactions of the cellular prion protein with amyloid-β, tau and α-synuclein oligomers are important in mediating the toxicity of these proteins in Alzheimer disease and Parkinson disease. Thefindings suggest a shared pathway that could be a therapeutic target common to multiple neurodegenerative diseases. • Jacob I. Ayers • & Stanley B. Prusiner

What is fatal familial insomnia? Fatal familial insomnia (FFI) is a very rare There’s an even rarer variant called sporadic fatal insomnia. However, there have only been FFI gets its name partly from the fact that it often causes death within a year of two of symptoms starting. However, this timeline can vary from person to person. It’s part of a family of conditions known as prion diseases. These are rare conditions that cause a loss of nerve cells in the brain. Other prion diseases include The symptoms of FFI vary from person to person. They tend to show up between the ages of 32 and 62. However, it’s possible for them to start at a younger or older age. Possible symptoms of early stage FFI include: • trouble falling asleep • trouble staying asleep • muscle twitching and spasms • muscle stiffness • movement and kicking when sleeping • loss of appetite • rapidly progressing dementia Symptoms of more advanced FFI include: • inability to sleep • deteriorating cognitive and mental function • loss of coordination, or ataxia • • excessive sweating • trouble speaking or swallowing • unexplained weight loss • fever FFI is caused by a mutation of the PRNP gene. This mutation causes an attack on the thalamus, which controls your sleep cycles and allows different parts of your brain to communicate with each other. It’s considered a progressive neurodegenerative disease. This means it causes your thalamus to gradually lose nerve cells. It’s this loss of cells that lead to FFI’s rang...

Overview Creutzfeldt-Jakob (KROITS-felt YAH-kobe) disease, also known as CJD, is a rare brain disorder that leads to dementia. It belongs to a group of human and animal diseases known as prion disorders. Symptoms of Creutzfeldt-Jakob disease can be similar to those of Alzheimer's disease. But Creutzfeldt-Jakob disease usually gets worse much faster and leads to death. CJD received public attention in the 1990s when some people in the United Kingdom became sick with a form of the disease. They developed variant CJD, known as vCJD, after eating meat from diseased cattle. However, most cases of Creutzfeldt-Jakob disease haven't been linked to eating beef. All types of CJD are serious but are very rare. About 1 to 2 cases of CJD are diagnosed per million people around the world each year. The disease most often affects older adults. Symptoms Creutzfeldt-Jakob disease is marked by changes in mental abilities. Symptoms get worse quickly, usually within several weeks to a few months. Early symptoms include: • Personality changes. • Memory loss. • Impaired thinking. • Blurry vision or blindness. • Insomnia. • Problems with coordination. • Trouble speaking. • Trouble swallowing. • Sudden, jerky movements. Death usually occurs within a year. People with Creutzfeldt-Jakob disease usually die of medical issues associated with the disease. They might include having trouble swallowing, falls, heart issues, lung failure, or pneumonia or other infections. In people with variant CJD, chang...