Do all prion diseases predominately affect the brain?

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It seems that way, but since they're just misfolded proteins it's strange for them to only attack the brain.
Prion diseases OR "transmissible spongiform encephalopathies (TSEs)" are a family of rare progressive neurodegenerative disorders that affect both humans and animals. They are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss, and a failure to induce inflammatory response.
(originate in brain, so, its common to say brain diseases).

The causative agent of TSEs is believed to be a prion. A prion is an abnormal, transmissible agent that is able to induce abnormal folding of normal cellular prion proteins in the BRAIN, leading to brain damage and the characteristics signs and symptoms of the disease. Prion diseases are usually rapidly progressive and always fatal.

A List of Prion Diseases
Listed below are the prion diseases identified to date. CDC does not currently offer information here on every prion disease listed.
Human Prion Diseases
Creutzfeldt-Jakob Disease (CJD)
Variant Creutzfeldt-Jakob Disease (vCJD)
Gerstmann-Straussler-Scheinker Syndrome
Fatal Familial Insomnia
Kuru
Animal Prion Diseases
Bovine Spongiform Encephalopathy (BSE)
Chronic Wasting Disease (CWD)
Scrapie
Transmissible mink encephalopathy
Feline spongiform encephalopathy
Ungulate spongiform encephalopathy  (+ info)

How can you develop immunity to prion diseases?

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You cannot develop immunity to prion diseases.

You cannot be vaccinated.
You cannot develop resistance by natural infection.

There are 5 prion diseases in humans - all progress to inevitable death. There are no treatments. I don't know what is responsible for the leg symptoms of the other respondent , above, but it was definitely not caused by a prion disease.  (+ info)

Can I get a prion disease from eating brains?

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I eat brains sometimes, but i was wondering. . . Can i get transmissible spongiform encephalopathy or other prion diseases from eating them? Or does cooking them make it safe?

A little concerned.
You can get BSE from eating any part of the infected cow or animal. The proteins effect the brain, but they are spread throughout the body! Makes me kind of scared to eat red meat.  (+ info)

What are prion diseases?

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it'll be better if u can give me a chemical perspective of the disease.
Prion diseases or transmissible spongiform encephalopathies (TSEs) are a family of rare progressive neurodegenerative disorders that affect both humans and animals. They are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss, and a failure to induce inflammatory response.

The causative agent of TSEs is believed to be a prion. A prion is an abnormal, transmissible agent that is able to induce abnormal folding of normal cellular prion proteins in the brain, leading to brain damage and the characteristics signs and symptoms of the disease. Prion diseases are usually rapidly progressive and always fatal.  (+ info)

Strategies for Treating Prion Diseases?

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At present what are the main strategies being looked at which may lead to treatments for prion diseases
Quinacrine, an oral drug used against malaria, has proven ineffective against prion disease, a deadly degenerative disorder of the brain, a study published by the journal The Lancet Neurology said Tuesday.

Doctors tested the drug among 107 patients with prion disease, who were assigned to a group that either took a 300-milligram dose of quinacrine daily or to another that did not take the treatment.

Seventy-eight of the patients died.


The death tally was somewhat lower in the quinacrine group, but after factors such as disease severity and type of disease were taken into account, there was no difference in survival.

The study was led by John Collinge, of the Prion Unit at Britain's Medical Research Council (MRC).

Prion diseases occur when a mutated form of the prion protein runs amok, destroying brain cells.

The term includes Creutzfeldt-Jakob disease (CJD), and a "variant" form that is the human equivalent of mad-cow disease, also called bovine spongiform encephalopathy (BSE).

Quinacrine was put to the test among human volunteers as it had been effective in lab-dish tests among mouse brain cells that had been infected with the rogue protein. The drug was "reasonably tolerated" among the patients, the study added.  (+ info)

Why can't prion diseases be cultured?

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I know that they can't be cultured, but WHY?
Who ever provides a referenced website, and a good answer, will be chosen as best answer! Thanks!
http://www.cdc.gov/ncidod/dvrd/prions/
http://www.cjdsurveillance.com/  (+ info)

What are the most greusome diseases/viruses?

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I'm thinking of things like necrotizing fasciitis, ebola, smallpox, prion disease, etc. What are a few more really greusome, terrible diseases or viruses?
Naegleria fowleri can invade the central nervous system via the nose, more specifically the olfactory mucosa and nasal tissues. The penetration initially results in significant necrosis of and hemorrhaging in the olfactory bulbs. From there, amoebae climb along nerve fibers through the floor of the cranium via the cribriform plate and into the brain. It then becomes pathogenic, causing primary amoebic meningoencephalitis (PAM or PAME). PAM is a syndrome affecting the central nervous system, characterized by changes in olfactory perception (taste and smell), followed by vomiting, nausea, fever, headache, and the rapid onset of coma and death in two weeks.

PS you can get this from hot tubs, so dont put your head under the water! There is a 3% survival rate because it is often very hard to diagnose before its too late.  (+ info)

How can prion (abnormally fold protein) can cause CNS damage? Why the disease is most known in sheep? ?

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Prion disease in animals is sheep scrapie. The disease form the sponge like aappearancein the brain. If the scrapie infected sheep is fed to cows, the mad cow disease develop. If a person eats poorly cooked beef, we can also be transmitted with mad cow disease.
This is a very complicated question. And there are some clarifications that should be made to statements in the text which follows the question.

First - prion disease in animals takes different forms depending on the species of origin (if the disease was transmitted) and the species which has contracted the disease. Prion disease in cows is called Bovine Spongiform Encephalopathy (otherwise known as Mad Cow Disease). Other animals like goats, deer, elk, moose, mink, some felines, and others can get prion disease. In humans there are several variants of prion diseases . Humans can become ill with Creutzfeldt-Jakob Disease (CJD), variant CJD (from eating 'mad cow beef'), Kuru, Fatal Familial Insomnia (FFI), and Gerstmann-Sträussler-Scheinker (GSS) disease.

Prion disease in sheep is indeed called Scrapie, and is partially characterized by microscopic sponge-like appearance in the brain.

Second - the transmission of Scrapie to cows has been documented experimentally, however it is unknown whether feeding cows sheep meat caused the Mad Cow epidemic of the UK, or whether this would happen in "real life" or non experimental conditions.

Third - eating poorly cooked beef does not conclusively increase your chances of contracting mad cow disease. Although prions are in very low concentration in muscle tissue or meat, they are very hard to inactivate. So cooking may not affect infectivity. The bottom line: in the entire world, just over 200 cases of humans becoming ill from this type of infection (vCJD) have been documented.

Finally - to answer the two questions:

A. How a protein can cause CNS damage
- The prion protein, when misfolded, is not in its normal cellular state. Also, the newly misfolded proteins aggregate and form into very stable 'clumps' called amyloid plaques. One could say that the human body does not know how to deal with the protein in this state, and normal cellular processes cannot break down the clumps. Cells in the immediate area die because normal processing cannot occur. There is a broad range of neural degeneration events that follow. Autophagy is a type of programmed cell death which is characterized by the formation of large vacuoles that eat away and break down cell parts. This process, when caused by the excess of prion clumps (amyloid plaques) occurring throughout the brain and CNS is as close as I can get to answering the question of how a protein can cause CNS damage.

B. Why the disease is most known in sheep
- I think this is because Scrapie is the most prevalent Transmissible Spongiform Encephalopathy. Many sheep accross the world (with the exception of New Zealand and Austrailia) die of Scrapie every year, whereas, the other diseases are quite rare. Also, Scrapie was the first TSE disease humans were really aware of. It was documented by farmers/vets in the 18th century.  (+ info)

How can a prion enter the human body....?

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I heard about these things, and i am interested how can prions enter the human body? Is that known? Can they be a part of some vaccine maybe( it sounds a bit stupid yeah.....in that case there would be much more cases of those diseases, as everyone is getting vaccinated against some diseases) ?
Prions are proteins. They are already in the body.  (+ info)

What is a prion and what does it cause?

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What is a prion and what disease(s) is it linked with?
AtomicHorse - A prion is a microscopic protein particle similar to a virus but lacking nucleic acid, thought to be the infectious agent responsible for scrapie and certain other degenerative diseases of the nervous system.
Prion diseases are often called spongiform encephalopathies because of the post mortem appearance of the brain with large vacuoles (holes) in the brain cortex and cerebellum. Probably most mammalian species develop these diseases. Specific examples include:
Scrapie: sheep
TME (transmissible mink encephalopathy): mink
CWD (chronic wasting disease): muledeer, elk
BSE (bovine spongiform encephalopathy): cows
Humans are also susceptible to several prion diseases:
CJD: Creutzfeld-Jacob Disease
GSS: Gerstmann-Straussler-Scheinker syndrome
FFI: Fatal familial Insomnia
Kuru
Alpers Syndrome
It is fascinating that a prion, which is not a living organism, can get into body cells and reproduce itself to cause deadly diseases. Prion research is a great field for future research.  (+ info)

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