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Scientists identify most lethal known species of prion protein

Scientists from the Florida campus of The Scripps Research Institute have identified a single prion protein that causes neuronal death similar to that seen in "mad cow" disease, but is at least 10 times more ...

Medical research created Feb 09, 2012 | popularity 4.8 / 5 (9) | comments 2 | with audio podcast

Making memories last: Prion-like protein plays key role in storing long-term memories

Memories in our brains are maintained by connections between neurons called "synapses". But how do these synapses stay strong and keep memories alive for decades? Neuroscientists at the Stowers Institute for ...

Medical research created Jan 27, 2012 | popularity 4.9 / 5 (15) | comments 1 | with audio podcast

Researchers find protein deposits linked to Alzheimer's disease behave like prions

(Medical Xpress) -- Researchers from the University of California have found that a peptide that forms deposits in the human brain and is thought to be responsible for the onset of Alzheimer’s disease, behaves in ways ...

Medical research created Jun 20, 2012 | popularity 5 / 5 (3) | comments 0 | with audio podcast report

Blood test for human form of mad cow disease developed

(Medical Xpress) -- Mad cow disease is serious business in the U.K., the human form, known as Creutzfeldt-Jakob after Hans Gerhard Creutzfeldt and Alfons Maria Jakob (CJD), who independently first described its existence ...

Diseases, Conditions, Syndromes created Jan 16, 2012 | popularity 4 / 5 (4) | comments 0 | with audio podcast report

Scientists identify first potentially effective therapy for human prion disease

Human diseases caused by misfolded proteins known as prions are some of most rare yet terrifying on the planet—incurable with disturbing symptoms that include dementia, personality shifts, hallucinations ...

Medical research created Apr 03, 2013 | popularity 5 / 5 (5) | comments 1 | with audio podcast

Proteins with mutations in 'prion-like' segments considered candidates for inherited forms of ALS

A multi-institution group of researchers has found new candidate disease proteins for neurodegenerative disorders. James Shorter, Ph.D., assistant professor of Biochemistry and Biophysics at the Perelman ...

Medical research created Mar 03, 2013 | popularity 5 / 5 (1) | comments 0 | with audio podcast

Prion protein hints at role in aiding learning and memory

Scientists from the University of Leeds have found that the protein called prion helps our brains to absorb zinc, which is believed to be crucial to our ability to learn and the wellbeing of our memory.

Medical research created Oct 16, 2012 | popularity 5 / 5 (4) | comments 0 | with audio podcast

Accused of complicity in Alzheimer's, amyloid proteins may be getting a bad rap

Amyloids—clumps of misfolded proteins found in the brains of people with Alzheimer's disease and other neurodegenerative disorders—are the quintessential bad boys of neurobiology. They're thought to muck up the seamless ...

Alzheimer's disease & dementia created Apr 03, 2013 | popularity 4 / 5 (1) | comments 1 | with audio podcast

Scientists show copper facilitates prion disease

(Medical Xpress) -- Many of us are familiar with prion disease from its most startling and unusual incarnations—the outbreaks of “mad cow” disease (bovine spongiform encephalopathy) that created a crisis in ...

Diseases, Conditions, Syndromes created Aug 10, 2012 | popularity 5 / 5 (4) | comments 0 | with audio podcast

New MRI technique may predict progress of dementias

A new technique for analyzing brain images offers the possibility of using magnetic resonance imaging (MRI) to predict the rate of progression and physical path of many degenerative brain diseases, report scientists at the ...

Neuroscience created Apr 10, 2012 | popularity 5 / 5 (1) | comments 0 | with audio podcast

New yeast prion helps cells survive

One of the greatest mysterious in cellular biology has been given a new twist thanks to findings reported in Science. Researchers at the RIKEN Brain Science Institute show that prions, proteins that transm ...

Medical research created Apr 23, 2012 | popularity 5 / 5 (1) | comments 0 | with audio podcast

Researchers discover possible key to degenerative nerve diseases

(Medical Xpress) -- Researchers at the University of Wisconsin-Madison and collaborators have discovered a powerful new protein in the eye of the fruit fly that may shed light on blinding diseases and other ...

Neuroscience created Nov 17, 2011 | popularity 5 / 5 (2) | comments 0 | with audio podcast

'Good' prion-like proteins boost immune response

(Medical Xpress) -- A person's ability to battle viruses at the cellular level remarkably resembles the way deadly infectious agents called prions misfold and cluster native proteins to cause disease, UT Southwestern Medical ...

Medical research created Aug 09, 2011 | popularity not rated yet | comments 0 | with audio podcast

The good side of the prion: A molecule that is not only dangerous, but can help the brain grow

A few years ago it was found that certain proteins, the prions, when defective are dangerous, as they are involved in neurodegenerative syndromes such as the Creutzfeldt-Jakob and the Alzheimer diseases. ...

Medical research created Feb 14, 2013 | popularity 4.7 / 5 (3) | comments 0 | with audio podcast

Protective prion keeps yeast cells from going it alone

Most commonly associated with such maladies as "mad cow disease" and Creutzfeldt-Jakob disease, prions are increasingly recognized for their ability to induce potentially beneficial traits in a variety of organisms, yeast ...

Medical research created Mar 28, 2013 | popularity 5 / 5 (1) | comments 0 | with audio podcast

Prion

A prion (pronounced /ˈpriː.ɒn/ ( listen)) is an infectious agent that is composed of protein. To date, all such agents that have been discovered propagate by transmitting a mis-folded protein state; the protein does not itself self-replicate and the process is dependent on the presence of the polypeptide in the host organism. The mis-folded form of the prion protein has been implicated in a number of diseases in a variety of mammals, including bovine spongiform encephalopathy (BSE, also known as "mad cow disease") in cattle and Creutzfeldt-Jakob disease (CJD) in humans. All known prion diseases affect the structure of the brain or other neural tissue, and all are currently untreatable and are always fatal. In general usage, prion refers to the theoretical unit of infection. In scientific notation, PrPC refers to the endogenous form of prion protein (PrP), which is found in a multitude of tissues, while PrPSC refers to the misfolded form of PrP, that is responsible for the formation of amyloid plaques that lead to neurodegeneration.

Prions are hypothesized to infect and propagate by refolding abnormally into a structure which is able to convert normal molecules of the protein into the abnormally structured form. All known prions induce the formation of an amyloid fold, in which the protein polymerises into an aggregate consisting of tightly packed beta sheets. This altered structure is extremely stable and accumulates in infected tissue, causing tissue damage and cell death. This stability means that prions are resistant to denaturation by chemical and physical agents, making disposal and containment of these particles difficult.

Proteins showing prion-type behavior are also found in some fungi and this has been important in helping to understand mammalian prions. However, fungal prions do not appear to cause disease in their hosts and may even confer an evolutionary advantage through a form of protein-based inheritance.

The word prion is a compound word derived from the initial letters of the words proteinaceous and infectious, with -on added by analogy to the word virion.

For more information about Prion, read the full article at Wikipedia.
This text uses material from Wikipedia and is available under the GNU Free Documentation License.

Related topics: protein