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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

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

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

Normal prion protein regulates iron metabolism

An iron imbalance caused by prion proteins collecting in the brain is a likely cause of cell death in Creutzfeldt-Jakob disease (CJD), researchers at Case Western Reserve University School of Medicine have found.

Alzheimer's disease & dementia created Mar 13, 2013 | popularity 5 / 5 (2) | comments 0 | 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

Scientists identify molecular system that could help develop potential treatments for neurodegenerative diseases

Scientists from the University of Southampton have identified the molecular system that contributes to the harmful inflammatory reaction in the brain during neurodegenerative diseases.

Neuroscience created Feb 21, 2013 | popularity not rated yet | comments 0

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

Brain inflammation likely key initiator to prion and Parkinson's disease

In a recent publication, researchers of the Computational Biology group at the Luxembourg Centre for Systems Biomedicine showed that neuro-inflammation plays a crucial role in initiating prion disease.

Parkinson's & Movement disorders created Nov 29, 2012 | popularity 5 / 5 (1) | comments 0

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

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

Designer compounds inhibit prion infection

(Medical Xpress) -- A team of University of Alberta researchers has identified a new class of compounds that inhibit the spread of malfunctioning proteins in the brain that cause lethal neurodegenerative diseases ...

Medical research created Jul 20, 2012 | popularity 5 / 5 (1) | comments 0 | with audio podcast

'Mad cow disease' in cattle can spread widely in ANS before detectable in CNS

Bovine spongiform encephalopathy (BSE, or "mad cow disease") is a fatal disease in cattle that causes portions of the brain to turn sponge-like. This transmissible disease is caused by the propagation of a misfolded form ...

Diseases, Conditions, Syndromes created Jul 09, 2012 | popularity 5 / 5 (1) | comments 0

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

Holstein with mad cow disease was lame, lying down

(AP) -- The cow that was recently discovered with mad cow disease through routine testing in California had been euthanized after it became lame and started lying down at a dairy, federal officials revealed ...

Diseases, Conditions, Syndromes created Apr 27, 2012 | popularity not rated yet | comments 1

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

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