Do Prions Exist?

"What are prions? The name prion was coined in 1981 by Dr. Stanley Prusiner to identify the agents that cause a novel type of fatal brain diseases. Bovine spongiform encephalopathy (BSE or mad cow disease), sheep scrapie and Creutzfeldt-Jakob disease (CJD) of humans are examples of prion diseases. In this essay, I will use the term prion instead of agent to denote the infectious particle that causes these diseases. The "protein-only" hypothesis is a controversial hypothesis that describes what prions are and how they reproduce. The discussion that follows is necessarily limited to only the most important evidence in support of the protein-only hypothesis due to space limitations."

www.pbs.org...

There is no science that this lump of protein does anything whatsoever. Prions are BS. Mad cow is a fake just like bird flu.............

President Bush was so concerned about prions that he made them "Select Agents" under the 2002 Bioterrorism Act. Now, it is an act of treason for US scientists to speak publicly about prions. So you won't be getting any argument from the scientific community in the USA.


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ed. bad wd

[edit on 14-11-2005 by soficrow]

How do you jump from the fact that there is scientific debate over the causes of these diseases to the conclusion that they are “fake?”

As the article you linked to points out, these diseases have been around and have been documented for a long time. They are not “fake.”

This is pretty ridiculous. Prions don't exist because some yahoo on PBS's website doesn't understand the mechanism. Please.

So it doesn't make sense to this person, it invalidates Prusiner's hypothesis? If this individual is so sure of him self, perhaps he should submit a letter to a journal refuting Prusiner's hypothesis... not posting stuff on the web.

Do you actually know anything about Prions other than what you've read on this site?

This individuals lack of understanding is perfectly demonstrated via the following statement
Nucleic acid does in fact carry the information necessary to construct the protein. This is not a dilemma at all. The issue of information is somewhat moot. The information is there and it's corrupt. The question is WHY is it corrupt. Despite what this PBS website might say. The answer has to do with energy minima. The prion proteins quite simply have more than one energy minima state they can occupy. It would appear that the non-TSE version is the preferred conformation, but the disease causing form is a secondary option that is likely harder to get to but orders of magnitude more stable. The information problem that this person describes is a problem only for him and his apparent lack of understanding with respect to the central dogma of biology...

Sofi... I am sure you've got some more concrete links you can add to this discussion.

Can you please point out the page on this copy of the Bioterroism Act of 2002 that details those claims?

[edit on 14-11-2005 by HowardRoark]

The constraints are established by the combination of Acts and Executive Orders - the main Gag Order is in the amendment to Executive Order 12958.

I covered most of these issues long ago, here:

U.S. Still Silencing Scientists


More supporting info:

* Interesting testimony before the Judiciary Committee:
commdocs.house.gov...
* Bill Summary and status:
www.congress.gov...:H.R.339
* Current text:
www.congress.gov...:4:./temp/~c108KV7HDS

“Mad cow as bioterrorism? Scientists worry that US gov't classification of BSE prions as ‘select agents’ could hinder research”
www.biomedcentral.com...

“US blocking prion research”
www.biomedcentral.com...

“US amends ‘select agent’ regs,” The Scientist, November 10, 2003. J.M. Perkel,
www.biomedcentral.com...

* Bioterrorism Preparedness and Response Act of 2002
www.aphis.usda.gov...

www.uspharmacist.com.../files/Feat/ACF2F0A.cfm&pub_id=8&article_id=43

Human Diseases

Kuru: Kuru is an obscure disease that was seen only among the Fore highlanders of New Guinea. This strange, fatal disease was marked by ataxia (loss of coordination) and often later by dementia. The affected individuals acquired kuru through ritual cannibalism; the Fore tribe had honored their dead by eating their brains.6 Because men in the tribe did not partake in the practice, the disease was almost exclusively found in women and young children. The disease annually afflicted about 1% of the total Fore population of about 30,000, some 300 cases per year. In the worst hit Fore settlements the incidence reached an astonishing 5% to 10%.

For those stricken with the disease, death came cruelly. The victims lost the ability to swallow and starved, or succumbed before starvation to pneumonia or to the deep, gangrenous bedsores they developed lying in their own urine or excrement.2 Fortunately, the practice of cannibalism in New Guinea has stopped, and kuru has virtually disappeared.

Creutzfeldt-Jakob Disease (CJD): A rare but deadly infectious human encephalopathy with an incidence of one to two cases per million people worldwide, Creutzfeldt-Jakob disease is uniformly fatal, with 90% of patients dying within the first year. CJD was first described in the early 1920s and classified with other degenerative brain diseases; its infectious nature was not established until 1968.

Clinically, the disease is manifested by a rapidly progressive dementia with associated neurological deficits and ataxia.7 Dementia advances rapidly, vision is impaired and muscle twitching becomes more obvious. Death occurs soon after. Histological examination of the brain at autopsy shows patchy spongiform changes in the cerebral hemispheres and a considerable loss of nerve cells in the occipital cortex. Fortunately, a new test is available which may be useful for diagnosing CJD when symptoms suggest that illness. The test will be especially helpful in distinguishing CJD from Alzheimer’s disease.

Creutzfeldt-Jakob disease occurs sporadically worldwide. It typically strikes those in their 60s. About 10%–15% of cases are inherited. Unfortunately, a small number of cases are spread during the process of treating another medical problem. The disease has apparently been transmitted by corneal transplantation, implantation of dura mater or electrodes in the brain, use of contaminated surgical instruments and injection of growth hormone derived from human pituitary glands.6 It has also been suggested that CJD might be transmitted by blood transfusion. Recently, amid mounting concern, blood bank personnel began asking donors if anyone in their family ever suffered from this brain disorder."

OK, so I lied. Yes Prions are real............Bush is hiding the truth from the public to keep panic down. All meat is tained............

Do you have a single study or anything remotely reasonable to suggest that they don't exist?

Science. 1991 Jun 14;252(5012):1515-22. Molecular biology of prion diseases.
Conversion of alpha-helices into beta-sheets features in the formation of the scrapie prion proteins. Proc Natl Acad Sci U S A. 1993 Dec 1;90(23):10962-6.
The cellular prion protein binds copper in vivo Nature 390, 684 - 687 (18 December 1997)
Prion diseases of humans and animals: their causes and molecular basis Annu Rev Neurosci. 2001;24:519-50.
Prion (PrPSc)-specific epitope defined by a monoclonal antibody Nature 390, 74 - 77 (06 November 1997)
Neurotoxicity of a prion protein fragment Nature 362, 543 - 546 (08 April 1993)
Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication. Cell. 1990 Nov 16;63(4):673-86.
Prion protein (PrP) with amino-proximal deletions restoring susceptibility of PrP knockout mice to scrapie EMBO Journal Vol 15, pp.1255-1264, 1996
Telling, G. C. et al. Evidence for the conformation of the pathologic isoform of the prion protein enciphering and propagating prion diversity. Science 274, 2079-2082 (19960
Peretz, D. et al. A conformational transition at the N-terminus of the prion protein features in formation of the scrapie isoform. J. Mol. Biol. 273, 614-622 (1997).
Butler, D. A. et al. Scrapie-infected murine neuroblastoma cells produce protease-resistant prion proteins. J. Virol. 62, 1558-1564 (1988).

I wouldn't say that anything in science is closed to debate, and certainly there is a lot of debate about prions, but it's on a slightly higher level than 'its government lies and bull#'

It also looks like the nova arguement is that, because there are genes in organisms that create prion proteins, then prior disease isn't protein only. Hardly that the prion proteins don't exist and don't cause disease.

Oh, the problem is much much bigger than tainted beef. We're talking contaminated water, soil, and plants too. How about the entire food supply, contaminated food processing facilities, and hospitals too? And don't forget those pesky microbes that inhabit our little planet - they've all got prions riding on their shoulders now.

Have you ever met a person with Mad Cow? It's real. Trust me. The worst thing about prions is they can't be killed, cause they're not alive!To say that prions don't exist is dangerous to say the least. If any of you want to be sure, that you'll never catch the BSE or the Mad Cow, here's a solution. Become a vegetarian (or a vegan if your paranoid of your dairy milk). I know, I know. It sounds preposterous. But if you wanna live, then is that what it's gonna come to?

Plus the E.U. subsidises the beef we eat for $2.50. That's more then what Third World Country people live on. I'm just turning vegatarian. Not because I care for animals. They don't care for me. It's because of the chemicals that they put in our meat. We expect it to be safe, but how do we know? Could BSE actually be a man-made travesty? Could all those chemicals that we pump into our mass-produced cattle cause Prions to exist? I look for an answer eagerly.
P.s Go vegetarian, you make a difference everyday.

[edit on 14/11/05 by MacDonagh]

Oh look, another lie about the Select Agent program from soficrow!

Who would have thought? Should I just link to my post about Select Agent sofi, or do you want to just go ahead and amend your lie for everyone here?

Not long ago, on PBS, there were 5 renown scientists who her breaking down minutest how the aids virus? works.
They shows prions and how they work.
They described prions as being like a bullet in the blood systen, they are simply giving out dis-ease, but since they are solid like a bullet, nothing so far has been able to penetrate the prion itself.Very interesting view to me.

A real meaningful discussion about prions. Sofi is right, prion have infected then entire food supply. For many years when research in high speed centrifuge was done many, many experiments were thought to have failed because lumps of "stuff" accumulated in the bottom of the test tubes. This "stuff" was washed down the biowaste drains and never given another thought. The "stuff" was mass amounts of prions. Bioresearch concentraited a fee mishapen proteins into bio-bombs.

CWD has now infected the elk/dear heard in Colorado, Wyoming, etc, etc. It is no surprise since one of the most active bioresearch labs is in northern colocrado. And in fact dumps its "treated" waste into the very river found to be the source of the CWD.

One prion can make thousands of duplicate malformed proteins in a process bioresearchers still don't understand.

THE ENTIRE FOOD CHAIN is already infected. Fish, Veggies, MILK.

Opps.........

[edit on 15-11-2005 by thermopolis]

I knew AIDS was linked to prions, but didn't know it was talked about publicly. Do you have a link or title? Sounds like a great program.

HIV is a lentovirus, a larger category of viruses than the retrovirus category which HIV also falls into. "Lento" virus means it is a "slow" virus which takes a long time to exert its harmful effects on the victim. Mad cow disease and Jakob Creutzfeld Disease are claimed to be slow "prion" diseases. A correlation seems possible on the basis of proteins that act over a long period of time and eventually become deadly.

I know the research shows a relationship - and that prions hitchhike on viruses, which seems to explain the mechanics. BUT - after this administration prevented unsanctioned US scientists from attending international the AIDS conference back in 2003?, all public discussion was squelched and dialogue was forced underground.

How bout I show you my references if you show me yours?

This should be trivially easy to demonstrate then, especially since the claim is that they pellet so easily. A centrifuge is not a particularly hard to get peice of equipment. Surely someone who beleives this can demonstrate it, or indeed, any professor with immuno-assay techniques can do this.

It's demonstrated all the time Nygdan - protein aggregation is the bane of certain manufacturing processes - and experiments. Well documented and commonly acknowledged.

Proteins misfold easily and aggregate when they do so. Ie., cooked eggs - the solid egg white is misfolded protein. The issue/question is - "When and how do these misfolded proteins become infectious?" ...At least one part of the answer is, "When they come into direct physical contact with similarly conformed proteins."


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