'Human remains link' to BSE cases

ping

An article like this should include a reference to the Fore Tribe in Papua New Guinea which suffered from Kuru (laughing sickness) for years. It was caused by the respectful eating of family members at the time of death. When the cannibalism stopped, the Kuru stopped.

I inadvertently left off any mention of the fact that Kuru is a prion disease and was (at least for awhile, maybe still) listed in the Guinness Book of Records as the world's deadliest disease.

MAPLE LEAF RAGE - How the BSE Businessmen are driving the Canadian cattle ranching community to extinction.

By Mark Purdey. 22/1/05

Cattle Battle.

The new year came in with a flurry of light snow that lay scattered across our hillside farm, blanketing out the muddy ruts of 2004 with an almost surreal white brilliance. But the purity of that morning was somehow tainted by a series of desperately sad emails from Canadian family ranchers. The news of two fresh cases of mad cow disease in Alberta, had clearly shocked the rural communities into a state of virtual suicidal despair. Following on from a year of US border closure that had virtually barricaded the country’s cattle sales, combined with the problem of a pile up of cows without sufficient feed, this latest event has penetrated deeper into the heart of Canadian rustic culture, driving a hard working vibrant community closer to the brink of extinction.

In real health risk terms, these two new cases of BSE do not warrant any need for alarm since it is little different from a couple of cows walking around with Alzheimers disease. In respect of the total number of BSE cases that Canada has experienced – a mere three cases to date – it’s completely insignificant. Great Britain has suffered 250,000 plus cases of BSE ( and we are still getting 250 cases per year ), which makes one wonder why there has been such a vehement degree of crisis-mongering over three BSE cows in a country as large as Canada.

My tiny farm in the UK has suffered three cases of BSE, yet thankfully we never had to endure the arrival of a slaughter squad of manic mandarins intent on taking out every animal for miles around, or zipped us up into space suits, closing down the farm and creating a biohazard zone. The disease had arrived with some purchased in pedigree cattle, yet never spread to our own home reared animals – despite 12 months of intermingling between the two groups. Our own BSE problem went away with the affected animals in the slaughter wagon, and has never reared its head again.

This same ‘non infectious’ pattern of BSE cause was exhibited by all of the 250,000 BSE cases that struck the British Isles. Since we have the benefits of a long epidemiological history on BSE behind us now, why do the global health authorities choose to deny the indisputable realities of this disease in preference for some ‘nightmare scenario’ ? – a hypothesis that is creating a multitude of major difficulties for so many for no good scientific reason.

In health risk terms, do these Canadian BSE cases actually warrant the virtual shutting down of the entire family farming community of Canada ? I think not.

North American governments need to question the underlying basis of the dogma on the origins of BSE .

The next question that the USDA and the Canadian governments need to be asking themselves, is how did these two cows develop BSE if they never eat tainted meat and bone meal feed – the supposed cause of BSE ? Unfortunately, the UK government could never face up to this most obvious and pressing question, which is strange, since over 43,000 cows which were born after the 1988 ban on this feed in the UK have still gone down with BSE. Furthermore, many European countries have now had more cases of BSE in cows born after their respective bans, than in cows born before their bans. Canada has now joined this ever growing list.

And since the UK have exported hundreds of thousands of tons of the MBM feed (from the 1960s to the 1990s ) to countries that have never had a single case of BSE , then perhaps it’s about time that we faced up to the obvious; that the feed did not cause BSE ?

R-Calf’s misguided action against the USDA will backfire, and end up shooting all US ranchers in the foot.

One of the main US cattle groups, known as R Calf, are not helping matters. In fact, their current actions to sue the USDA for rightly planning to lift the block on Canadian beef imports across the border is misguided. R Calf’s actions will ultimately shoot their own ranching businesses in the foot. They are merely feeding the myth of the mad cow "crisis", which will devour them all in the end.

But since R-Calf are trying to blockade Canadian beef imports on the basis of an unproven, bogus argument – that the hyperinfectious protein-only contagion contained in BSE affected beef (albeit the three Canadian cases ) will pose a grave threat to US livestock and human health - then they are going to establish this mindset within the ranks of their own marketplace. But R Calf’s actions will backfire because the moment another case of BSE appears on the US side of the border, then the US rancher will have to face the full consequences of Calf’s mythmongering.

When I lectured to a group of these folk at Billings in Montana, one of the questions from the floor raised the issue of a live cow / infected feed study conducted in the UK, as though this work had provided the ultimate evidence upon which the current hyperinfectious paranoia of the global health authorities towards BSE is based.

Single unpublished experiment represents the sole basis behind the global gospel on BSE.

The whole global policy on BSE has been forged on the basis of a single, unpublished, non peer reviewed experiment that has purportedly involved the successful induction of BSE in 11 of 16 cows that had been fed varying oral doses (1g, 10g, 100g, 300g) - of brainstem homogenate taken from BSE affected cows.

Firstly in the real world cattle didn’t eat homogenate at all, let alone in a neat form like this. They ate MBM feed, a tiny fraction of which may have contained bse prions. This material would have been vastly ‘diluted’ by the mass of bulking nutriment used in feed manufacture. Therefore the experiment didn’t replicate either the dosage that an ‘on-farm’ cow would be likely to consume or the exact form and context of the dose.

But even if transmission was successful in these trials with some animals developing clinical BSE, we must remember that transmission does not necessarily imply an infection. You could merely be transmitting a toxic, heat resistant metal microcrystal that seeds the growth of an aberrant metal protein crystal aggregation once it gets implanted into the brain of a new host. I believe is the real cause of BSE.

But the UK government’s account of this all important wee study seems to vary dramatically – depending upon who is being told about it. The fact that these trial cows had been purchased off the ordinary British farm (And remember, BSE is probably seeded in early life, perhaps even during ‘in utero’stages ) indicates that these studies were scientifically flawed from the outset. And the small number of cows involved also invalidates the usefulness of the trial – whatever the outcome.

But the fact that the whole world has designed its BSE policy on the back of such an insignificant, unpublished study is curious , to say the least. Whenever governments have called their opposing forces, to provide evidence at public inquiries ( such as myself ), they rightly deem it imperative that any evidence submitted to the hearings must have already been profiled in the peer reviewed academic literature. But, true to form, it is one set of rules for the Establishment, and another, far more stringent set for their opponents.

Furthermore, the UK government has been giving out contradictory statements on both the protocols and timing/dates of this study. For some Canadian rancher friends, the Czar family, had written to the UK government asking about these tests, yet were told in the response that this experiment was still in progress ( and only a total of ‘ten cows’ involved ! ) and would be published shortly. But, seven years ago, a very reliable government official , Mr Tom Eddy, had informed my brother, Nigel Purdey, of these live cow tests and how they already had been completed with positive results. Again, no peer reviewed publication can be located. Nine months ago, the government informed me that these trials were complete and the results had been submitted to the EU commission. But in the absence of any journal publication, what are we to believe ?

UK Government has a track record of disseminating misinformation to North America.

Unfortunately, I have to say that the UK government exhibits a track record of disseminating misinformation into the public domain – and North America has become their latest victim in the propaganda campaign over the true cause of BSE. For the UK gov have an awful lot to hide in order to dodge a liability issue here.

In this respect, North American government officials would do well to question the validity of the statements in the Gabriel Horne Report on the Origins of BSE. A report that has been spun across the breadth of America / Canada and hailed as a flagship publication which charters the gospel according to the UK government on all issues surrounding BSE. But in truth, we are dealing with a document that uses disinformation and misrepresentation, whilst making a judicial selection of the data in order to funnel the outside world into the UK government’s agenda on BSE. Despite being authored by several "expert" Professors, the work is anything other than scientific.

For example, in order to persuade the US / Canadian governments to discard the worth of my own research data, they have promoted the Horne report which blatantly misleads the reader over the start / finish date of compulsory organo phosphate treatments for the control of warble fly – where , in truthful accord with the UK Animal Diseases Act and "The Warble Fly Order England and Wales 1982 " it first became compulsory to treat UK cows with exclusively high dose concentrations of systemic acting chemicals on a 2x per year basis in 1982.

Yet, in order to discredit my hypothesis – which proposes that BSE causation is associated with exposure of cattle to these organo phosphate nucleating agents - the UK gov have been promoting totally bogus statements via the Gabriel Horne report which alleges the following " since the majority of cows which developed BSE were born after 1982 [ true ], and the use of organophosphates for warble control had ceased by 1982 [false], then how can BSE be associated with exposure to these organo phosphate warble fly compounds". Yet in reality, the opposite is the truth, since compulsory use of these chemicals had actually first started in 1982, which entirely supports my position on the cause of BSE. In this respect, I find it hard to believe that the British Dept of Agriculture cannot remember the start date of their very own Act - which they themselves had formulated for the UK parliamentary Statute book .

The Nucleator; a scientific explanation for metal microcrystals as the cause of BSE

The Uk government are obviously worried about the threats of any potential liabilities that might arise from any further validation of my work – particularly if the primary role of organo phosphates in the pathogenesis of BSE continues to be proven. Thus, in accord with my theory, it was the government’s compulsory treatment of bovines with these systemic acting organo phosphate nucleating agents, used at exclusively high dose rates in the UK, that lies at the centre of the cause of this disease. For organo phosphate oil based formulation had to be poured along the spine line of the cow – just millimetres from the central nerves where the BSE disease process is first initiated . This oil based systemic formulation delivered the phosphorus ‘nucleator’ across the skin of the treated cow, and into the central nerves; thereby seeding the growth of aberrant metal-prion protein-ferritin crystals – the so called fibril structures that hallmark the neuropathology of the mad cow brain.

These rogue crystals are piezoelectric by nature, and therefore compromise the ability of the contaminated individual to deal with sound waves in the normal manner. This disrupts the homeostasis of electrochemical signalling in the brain, and sets in train a chain reaction of free radical mediated spongiform neurodegeneration - the deadly melt down in the cow’s brain.

But the scandal of deception and secrecy around the origins of BSE does not stop there. Compulsory Warble Fly treatments were not the only source of the highly toxic organo phosphate compounds to permeate the shores of the British Isles. For this group of compounds were first synthesised as nerve gases by Hitler’s military arsenal, and were actually used by Saddam to run gas attacks on the misfortunate Kurds – with apocalyptic consequences.

During the 1980s and 1990s, Great Britain overdosed itself with a wide array of organophosphate compounds used for a myriad of different purposes.

If you go down to the woods today, you can be sure of a big surprise.

Few in North America will know about the quaint little English village of Queniborough, and how it has experienced the most intensive cluster of variant CJD in the world. Furthermore, British secrecy has been so closely guarded, that few, if any folk in Queniborough itself seem to know that their local munition factory " The Queniborough Ordnance Depot" was a major producer of chemical weaponry during world war two.

For Winston Churchill had ordered that hundreds of thousands of tons of phosgene, mustard gas and other undisclosed chemical munitions, detonators and triggers to be assembled on this massive 130 acre site, and stored around the lanes of Queniborough to evade the German bombing sorties.

The loaded bombs were later dispatched to various woodland depots close to the bomber bases all the way down the east coast of England, from Northern Scotland to Kent, in readiness to blitz every German city with chemical munitions , if it looked like the Allies were going to loose the war. Fortunately they were never used, although hundreds of thousands of tons of these munitions ( including the organo phosphates ) were taken out by boat to the North and Irish seas, and then dumped onto the sea bed. But once sufficient years had passed for the canisters to start corroding, the sinister legacy of these evil substances has come back to haunt us in the form of BSE and vCJD – and possibly other conditions as well.

When meat and bone meal was ironically banned in 1988, the animal feed merchants increased the usage of other sources of protein, such as fish meal, which had been sourced from the very areas of UK waters where these nerve gases had been dumped. The rates of BSE then escalated right up until 1993, at which point the epidemic peaked at 3,500 cases per month, and then gradually went into decline up to present day. In this respect, the consumption of a plate of good olde English fish and chips could have had more to do with the cause of vCJD than beef.

Given that every victim of vCJD in the Queniborough cluster had had direct contact with the former site of this munitions factory in their village, and the other rural clusters of vCJD had emerged in villages which adjoin the woodlands and former airfields where these ‘persistent’ munitions were stored, immediate attention should be directed towards unravelling this riddle of TSE cause.

It is interesting that the majority of vCJD victims had a tendency to be involved in outdoor lifestyles; where woodcutters, farm workers, horse riders , kennel workers ,etc, had developed vCJD – the very people who were eating the local game birds/rabbits and wild fruit / nuts, etc, that had been grown from the ecosystems of these contaminated woodlands.

But, true to Establishment form, the research investigation into the cause of vCJD is conveniently closed on the ‘beef cause’ – despite the flaws in the theory. The local health researcher from Leicester was knighted for successfully shifting the blame of the Queniborough vCJD cluster onto the innocuous butchery practises of the local Butcher – hardly a plausible explanation for the unique emergence of such a rare disease cluster, when these butchery practises are carried out at every rural butcher’s shop across the UK !

Likewise, another local butcher at Lymphstone village in Devon found himself dragged into the firing line of blame for another collection of vCJD cases. This was particularly stupid because one of these victims was billeted at the local military marine camp on the edge of the village, and the marines invariably purchase their meat supplies from the large wholesale consortiums. One meal of meat at the marine camp would have bought out the entire stocks of the village butcher.

Cluster buster.

I have visited and sampled every well renowned cluster of TSEs across the world, and in each location I have identified excessively high levels of one or other of the types of metal microcrystal – strontium, barium, silver or phosphorus – that act as nucleators in the biosystem and seed the growth of metal-prion protein- ferritin crystals. Each different species of metal generates a different strain of spongiform disease.

But in any truly impartial, free thinking society, one would have thought that the existence of this unique toxic metal ‘common denominator’ that I have observed in each cluster ecosystem warrants an immediate public health investigation. But despite publication of some of my data in peer reviewed science journals, this important observation is being ignored.

Nonetheless, the academic community and gullible mass media keep reporting the latest experiments from the lab benches of Prusiner et al; where misfolded prions injected into misfortunate lab animals will produce TSEs as a direct result. This is highly interesting research, but the misfolded prions are a secondary factor in the cause of TSEs. They are not the primary initiators. Prusiner and others know only too well, that they had to create their first generation misfolded prions in the lab in the first place. This transformation from healthy prion protein to misfolded prion protein did not simply happen on its own accord. For many scientific teams have produced these misfolded prions in the lab, but they have had to expose them to sound waves and metals / chemicals in order to induce a stable conformational alteration of the prion protein – the very prerequisites that I have been hypothesising to act as the primary environmental initiators that cause TSEs.

Yet these academics are too steeped in the world of lab technology to make a small creative leap in their thinking, and project the ‘in vitro’ initiators that have been involved in their lab experiments across to the ‘in vivo’ context of the open environment. The real world of the living cow – where a cocktail of chemicals and sonic shock waves are all part and parcel of our modern complex environments. This is, I believe, why we are seeing so many new diseases, like BSE, emerging. These environmental studies are all published in the scientific literature, and readily accessible through Pub Med database.

So why do the Establishment ignore the fact that every case ( I repeat every case ) of TSE from the remotest corner of the earth is correlating with some mode of exposure to the metal microcrystal nucleating agents - usually associated with the production, storage, incineration, testing of munitions. The reality of these observations cannot continue to be denied indefinately.

It is not as though my hypothesis was conceived during a whimsical session on a bar stool - like some theories. It was carefully developed over 15 years of intensive global travel, scientific analyses, local discussion / observation of the real world environment in these unique TSE cluster hotspot locations around the world.

For example, The Canadian clusters of TSEs along the Albertan / Saskatchewan borders, all relate to the atmospheric discharge of munition microcrystals from the exploding ordnance at the Cold Lake air weapons range and Camp Wainwright shelling range - or in the regions where, not so long ago, NATO went "on safari" to test chemical weapons on the wild animal populations ( deer, etc ) in that area.

There is also the case of the late Senator of the Isle of Guam who had died of CJD, who had accidentally unearthed dumped US ordnance on farmland adjacent to his family home prior to contracting CJD.

Not that far from Guam, the famous Fore tribe who thrived in the remote highland region of Papua New Guinea, were supposed to have contracted a type of CJD called kuru through their cannibalism. But, in truth, the Fore folk had accidentally exploded bombs whilst scavenging crashed USAF bombers that had been shot down over their territory during world war two. By the 1950s, the survivors had all developed the Kuru syndrome. This was the exclusive event that had caused this exclusive outbreak of CJD in Papua New Guinea. Nevertheless, this cluster was blamed on the tribe’s cannibalistic practises by US military medical researchers, despite the other 98% of the population across New Guinea, which practise cannibalism in the same way as the Fore, remaining Kuru-free.

Having been sceptical of the mainstream propaganda on the cannabalistic cause of this cluster, I have always wondered what substances were in the exploding bombs that had exclusively affected that area. My recent analyses of the soil in those highland craters has confirmed my suspicions.

The Nerve Agents.

The strange thing about the current situation in the Canadian cattle battle is that nobody in authority seems to care about the plight of their own rural community; particularly ironic, since these communities provide a key role in propping up the Canadian economy. And for the few who actually get CJD, nobody seems to do much for them either. They are just told that the disease has no cure, and are sent home to die.

Stranger still, is the total betrayal of the Canadian government ( or any government for that matter ) towards the best interests of their own people. Their indifferent stance towards the damages that have been caused by the politics of BSE is most strange, particularly when those damages only exist as a direct result of the way that BSE has been drummed up into such a major political, cultural and economic threat, when all along, the pure science behind the story indicates that this disease is not actually infectious in the true sense of the word.

Whilst we clearly need to investigate and eradicate this horrendous disease – simply because of the terror that it must impose upon its poor victims - we can only begin to achieve this by identifying and accepting the disease’s true cause.

But the official indifference that has been adopted by the Canadian Establishment towards its ailing rural communities is rapidly reversed into a state of enthusiastic co-operation when you witness them launching any kind of venture with the multinational corporates. Yet I believe it is the corporates who are forging a lucrative business out of BSE. They need people to be scared of BSE, in order to create a climate where they can market their multi billion pound packages of ‘live cow BSE tests’, BSE-resistant GM cows, or, better still, replace the world’s ‘dangerously infected’ meat and milk protein supply with their own genetically modified package of chemically grown, sterile GM arable protein crops (soya, etc) – guaranteed to be free of ‘dangerous’ pathogens .

In this respect, the Canadian mandarins are feeding the very multinational monster that is draining the blood from their own people.

The Multinational policy for global take over of the protein supply is being exercised by a handful of key individuals from the corporate underworld who have installed themselves into prime positions of power in virtually every country. In Canada, their field officers have back-scratched their way into the upper echelons of the Canadian Agri-world – the deer and cattle associations – where they are representing anything but the best interests of their own membership. It’s much the same here in the UK, where the corporations have installed their own double agents into the UK government’s civil service. They operate on the principle that if you place your people onto both sides of the chess board, then you will be sure to win the game.

And it is all but a game, a mere money game to them. But to the ranchers, it represents generations of hard work, intuitive wisdom and a lifestyle that requires an immense degree of inner integrity to sustain. Are we prepared to sit back and watch a chunk of our agricultural heritage going down the pan, merely to appease the short term profits of a handful of global tycoons – all for the purchase of products for which we have no need.

In this respect, the root of the problem seems to stem from the fact that too many vested interests have become involved in the Business of BSE. The resulting commercial pressures have corrupted the science, making it hard to expect any hard scientific facts to carry much weight with the government policy makers and advisors. The government departments are simply not interested in anything that challenges the commercial master-plan.

The evidence for this is out there. For a battery of ‘Bio’ companies are working up their marketplace in Canada right now. Most of these international based companies are connected back to one of the leading global corporations, who have been only to keen to keep the main governments hoodwinked on the hyperinfectious ‘health risks’ posed by BSE. This is aimed at coercing governments to give the ‘nod’ to the mass marketing of their four dollar per cow live BSE test, essentially so that it implicates every single rancher across North America in buying their test - not to mention their scheme to unleash GM prion protein knock out designer cows (guaranteed BSE resistant ) onto the farm scene.

This indicates that there is a great deal of money at stake for these companies. But what they have overlooked is the overall effect of their sustained campaign of scare-mongering and its negative repercussions on the economic status of the farming community at large. The critical swathe of Media words against livestock farmers over recent years has been sufficient to impoverish our farmlands.

It is therefore hard to see how the corporates will be able to market their 4 dollar per cow BSE live test, since the impact of their propaganda to get governments to run with their tests has ironically ended up rendering the poor old cow totally worthless .

One way forward is to plead with our governments that they return to the days when they acted more independently of commercial interests. We need them to call into question all of the junk science that has been laid down about the origins of BSE to date, and get them to reopen a truly independent investigation into the origins of this complex disease.

It seems unfair that the very companies who have been allowed to create BSE through the sales of their organo phosphate insecticides / war gas prerequisites, are now out and about looking for further revenue to make out of the "crisis" itself - by offering a commercial solution to the very problems that their products had created in the first instance.

I think I saw you mention that you have a canadian ping list?

Would you mind pinging to post 5 please?

Thank you very much. Using your search terms 'Fore Tribe in Papua New Guinea which suffered from Kuru' pulled alot more information!

CJD was found in, I believe it was 5 people, in the Twin Falls area of Idaho last fall that had died. A cluster of people dying from this is rather disconcerting.

North American governments need to question the underlying basis of the dogma on the origins of BSE.

Yes. I think this is a bit too close to junk science. Neural degenerative diseases are very poorly understood on all levels.

Blaming it on cows or now some story about human remains followed by the mass slaughter of animals strikes me as similar to a modern day blaming something madness on witches or doing a ritual to stave it off.

This was a very informative article.

Could you expound more on what these prions emanate from? Is it just munitions or is it also in chemicals that are used to spray cattle to eradicate lice/flys, etc.? Is it in phosphorous based fertilizers?

-

http://www-micro.msb.le.ac.uk/3035/prions.html

Prions do not have a nucleic acid genome. It seems that a protein alone is the infectious agent. The infectious agent has been called a prion. A prion has been defined as "small proteinaceous infectious particles which resist inactivation by procedures that modify nucleic acids". The discovery that proteins alone can transmit an infectious disease has come as a considerable surprise to the scientific community.
Prion diseases are often called spongiform encephalopathies because of the post mortem appearance of the brain with large vacuoles in the 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

Humans might be infected by prions in 2 ways:

1. Acquired infection (diet and following medical procedures such as surgery, growth hormone injections, corneal transplants) i.e. infectious agent implicated.

2. Apparent hereditary mendelian transmission where it is an autosomal and dominant trait. This is not prima facie consistent with an infectious agent.

Empasis:

>>>Kuru is the condition which first brought prion diseases to prominence in the 1950s. Found in geographically isolated tribes in the Fore highlands of New Guinea. Established that ingesting brain tissue of dead relatives for religious reasons was likely to be the route of transmission. They ground up the brain into a pale grey soup, heated it and ate it. Clinically, the disease resembles CJD. Other tribes in the vicinity with same religious habit did not develop the disease. It is speculated that at some point in the past a tribe member developed CJD, and as brain tissue is highly infectious this allowed the disease to spread. Afflicted tribes were encouraged not to ingest brain tissue and the incidence of disease rapidly declined and is now almost unknown.<<<

>>>Is it in phosphorous based fertilizers?

I couldn't find too much on that one. This article talks about that though.

Mad Cows in the Garden

Home

Hundreds of thousands of cows infected with mad cow disease (BSE) have been destroyed in England, and the British government believes that 10 people have succumbed to a human form of the disease as a result of eating infected beef. Last year, researchers concluded that it was possible for mad cow disease to be passed through beef bone and marrow, and the government subsequently banned the sale of most cuts of beef on the bone.

Bone meal is commonly used as a fertilizer in agriculture, especially in organic gardening. Is it possible that people could become infected with mad cow disease through contact with bone meal, either as they apply it to their gardens, or through ingesting residues on vegetables? Could the infective agents, called prions, be absorbed by plants and transferred to humans who consume them? No one knows for sure, but the British government has already banned the use of bone meal as a fertilizer in commercial agriculture.

The U.S. Department of Agriculture (USDA) contends that there is no need to worry about mad cow disease in this country, since no cases have turned up in U.S. cattle. Yet, some experts believe that a variant of the disease, perhaps just as deadly, may already be widespread. Also, infected bone meal may be imported from other countries with the disease.

To be on the safe side, experts recommend that gardeners handling bone meal wear masks and gloves, and take care not to become exposed to the substance through an accidental cut or splinter. Many high-phosphorous alternatives can substitute for bone meal, such as rock phosphate and soft rock phosphate.

[Editor: In a recent PBS special on mad cow disease, researchers found that prions were not harmed when buried in the ground for three years. In a recent conversation I had with a university plant scientist, he said that it was theoretically possible for prions from contaminated fertilizers to be absorbed by plants. Given these facts, it might be prudent to avoid vegetables grown with bone meal until more is known.]

In related news: Microbiologists at the Environmental Protection Agency (EPA) believe that the processed sewage sludge commonly sold as fertilizer for home gardening may contain disease-causing organisms such as Salmonella. Apparently, bacteria are able to hide in clumps of sludge, going undetected by standard tests for contamination. People who accidentally ingest small bits of sludge clinging to unwashed hands or improperly cleaned vegetables may become ill. Based on information in: Organic Gardening, Mar 1997; Science News, 2-28-98

Kuru: The Dynamics of a Prion Disease

Dr. Bindon

Introduction

An elusive and unknown disease appeared in New Guinea in the early 1900’s. By the 1950’s anthropologists and government officials reported that this disease termed kuru was rampant among the South Fore. The South Fore were identified by Australian government officials in the 1950’s as a single census division consisting of approximately 8,000 individuals within the Okapa Subdistrict (Lindenbaum, 1979). Observe the following maps in order to see the exact location of the South Fore.

First Map

Second Map

This particular group was partaking in ritual acts of mortuary cannibalism, and this conduct was later held to be responsible for the transmission of the fatal kuru epidemic. This distinctive aspect of the illness made it even more fascinating to the various Western scholars who devoted their time to conquering it. Many efforts have been made to understand and describe kuru, and the knowledge of the dynamics of the disease has continued to grow, even though the disease all but disappeared in New Guinea with the termination of cannibalism. The pathology and symptoms of kuru are of specific interest here, as well as a comparison of kuru with other prion diseases. Scientists have now identified kuru as a prion disease. Understanding the structure and replication of the prion is crucial to interpreting the dynamics of kuru and several other prion diseases, which exist today. The onset of kuru led to a study of an unfamiliar disease that has lasted almost five decades. This particular disease serves as an example of the procedures scientists undergo in order to understand and appreciate all of the aspects of a disease and how potential therapies and solutions can be found.

Kuru Among the South Fore

Kuru is a neurodegenerative disorder that surfaced among the South Fore of New Guinea, and the dynamics of this disease have been explored by various scholars. Lindenbaum worked with the South Fore and studied the kuru disease. Zigas worked in New Guinea, and Gadjusek also traveled there in 1957 to study disease patterns in primitive and isolated populations (Gadjusek, 1996). Lindenbaum, Zigas, and Gadjusek were all crucial to explaining the marked, specific properties of kuru to the rest of the world.

The kuru epidemic reached its height in the 1960’s (Lindenbaum, 1979). Between 1957 and 1968, over 1,100 of the South Fore died from kuru (Lindenbaum, 1979). The vast majority of victims among the South Fore were women. In fact, eight times more women than men contracted the disease (Lindenbaum, 1979). It later affected small children and the elderly at a high rate as well. This is to be expected, since women were the prime participants in mortuary cannibalism (Lindenbaum, 1979). It is currently believed that kuru was transmitted among the South Fore through participation in such cannibalism. Upon the death of an individual, the maternal kin were in charge of the dismemberment of the corpse (Lindenbaum, 1979). The women would remove the arms and feet, strip the limbs of muscle, remove the brains, and cut open the chest in order to remove internal organs (Lindenbaum, 1979). Lindenbaum (1979) states that kuru victims were highly regarded as sources of food, because the layer of fat on victims who died quickly resembled pork. Women also were known to feed morsels such as human brains and various parts of organs to their children and the elderly (Lindenbaum, 1979).

Misinterpretations of Kuru

Scholars who first studied the disease among the South Fore initially had two major misconceptions concerning the nature of the disease. They first incorrectly postulated that it was a genetic disorder. After this possibility was ruled out, scientists next asserted that kuru was the manifestation of a slow virus. Genetic disorders can be fully understood through application to population genetics. Mutations provide variation and fuel natural selection. A genetic disorder is one that is caused by a mutation that is passed on to subsequent offspring. Since kuru had a tendency to occur among family members (Lindenbaum, 1979), the original notion that it was a genetic disorder seems somewhat appropriate. This possibility was eventually ruled out, because kuru was too common and too fatal (Lindenbaum, 1979). A completely lethal genetic disorder would drastically reduce the fitness of a population. Sooner or later it would die out of the gene pool. This fact led scholars to seek additional possible explanations to describe the dynamics of the disease.

Studies on chimpanzees injected with brain material from a victim led scientists to believe the agent was a slow virus, because the chimps developed a very similar condition after a long incubation period (Gadjusek et al., 1966). Gadjusek was responsible for conducting these tests on chimps. He defined a slow virus as a viral disease with an abnormally long incubation period (Gadjusek et al., 1966). In humans, kuru had an incubation period with a minimum of two years and maximum of 23 years (Lindenbaum, 1979:26). Gadjusek’s results also confirmed the infectious, transmittable nature of the prion. Mestel (1996:185) writes, "Since then, his [Gadjusek’s] team has shown that CJD [Creutzfeldt-Jakob disease] and GGS [German-Straussler-Scheinker syndrome] are also infectious..." With kuru, there was no evidence of an immune response or an antibody. It will become evident later that both of these hypotheses were incorrect. For now, the specific symptoms of kuru are relevant in gaining a more complete understanding of the disease as a neurological disorder.

Symptoms of Kuru

Gadjusek studied kuru, and he found the condition of kuru victims to be an upsetting sight. He explains, "...to see them, however, regularly progress to neurological degeneration in three to six months (usually three) and to death is another matter and cannot be shrugged off" (Gadjusek, 1996:10). Gadjusek (1973) reported three main stages in the progression of symptoms. The first stage is called the ambulant stage, and it includes unsteadiness of stance, gait, voice, hands, and eyes; deterioration of speech; tremor; shivering; in- coordination in lower extremities that moves slowly upward; and dysarthria (slurring of speech) (Gadjusek, 1973). The second stage is also known as the sedentary stage, and Gadjusek (1973) defines it with the following symptoms: patient can no longer walk without support, more severe tremors and ataxia (loss of coordination of the muscles), shock-like muscle jerks, emotional lability, outbursts of laughter, depression, and mental slowing (it is important to note that muscle degeneration has not occurred in this stage, and tendon reflexes are usually still normal) (Gadjusek, 1973). The third stage is the terminal stage, which is marked by the patient’s inability to sit up without support; more severe ataxia (loss of muscle coordination), tremor, and dysarthria (slurring of speech); urinary and faecal incontinence; difficulty swallowing (dysphagia); and deep ulcerations appear (Gadjusek, 1973). Cerebellar dysfunction is the cause of these conditions. Symptoms are generally common among prion diseases, as a comparison with Creutzfeldt-Jakob disease will demonstrate.

Comparison to CJD

Creutzfeldt-Jakob disease displays striking similarities to kuru in regards to symptoms displayed and organ damage (mostly to the brain). Comparisons and parallels are evident between these two prion diseases. By inspecting an in depth case study of CJD from Massachusetts General Hospital, it is possible to gain a more complete understanding of prion diseases.

At age 47, a woman feeling depression sought professional help at Massachusetts General Hospital (Scully et al., 1993). She became hypoactive, noticed impairment of her recent memory, and had urinary incontinence (Scully et al., 1993). Within a few months she became dizzy and had an unstable gait (Scully et al., 1993). At this point a computed tomographic scan (CAT scan) of the brain showed slight cerebral and central atrophy; delusion began to set in (Scully et al., 1993). According to Scully et al. (1993), by age 50 the patient’s cranial-nerve functions were still normal, as well as motor power, sensation, and coordination. The next symptom to appear was the occurrence of inappropriate laughter, and her replies to questions became irrelevant and incorrect (Scully et al., 1993). Mild tremor was noted, although the cranial-nerve functions, strength, coordination, and sensation were still intact (Scully et al., 1993). At this time another CAT scan was performed, and the results were the same as the year before (Scully et al., 1993). Within a week after this scan, she was readmitted with shaking spells (Scully et al., 1993). There was a constant alteration between laughing and crying, but reflexes were still normal (Scully et al., 1993). By the age of 51 and a half years, her speech had deteriorated rapidly, and a new CAT scan showed marked cerebral and cerebellar atrophy (Scully et al., 1993). According to Scully et al. (1993), gradual deterioration continued up until her death four months prior to her fifty-fourth birthday.

Important similarities occur between Creutzfeldt-Jakob disease (CJD) and kuru. Both prion diseases cause tremor and inappropriate laughter. Depression was expressed early in CJD and in stage two of kuru. Unsteadiness in gait and sporadic muscle jerks were observed in both ailments. Dysarthria occurred in kuru during the initial stages of the diseases and in CJD more towards the end, and the exact same situation is seen with the condition of urinary incontinence as well.

The Prion Protein

The exact nature of kuru perplexed scholars for decades after the discovery of the ailment, until Prusiner identified and defined prion diseases in 1982 (Prusiner, 1995). Prusiner (1991) classified a prion as an infectious particle composed of a protein that causes neurodegenerative disorders. According to Cashman (1997), prions are infectious agents by biological and medical criteria. However, they are also fairly unique, and properties of prions differ from those of conventional microbes. All known prion diseases are fatal. Such diseases are often called spongiform encephalies, because they frequently cause the brain to become spongy and riddled with holes (Prusiner, 1995). Well known prion diseases include scrapie, bovine spongiform encephalopathy (mad cow disease or BSE), and Creutzfeldt-Jakob disease (CJD). Less well-known prion diseases include the following: transmissible mink encephalopathy, chronic wasting disease, feline spongiform encephalopathy, exotic ungulate encephalopathy, German-Straussler-Scheinker syndrome (GSS), and fatal familial insomnia (Krakauer et al., 1997). Of these infirmities, four affect humans: Creutzfeldt-Jakob disease, Gertsmann-Straussler-Scheinker syndrome, fatal familial insomnia, and kuru. The most common form of prion disease is scrapie, expressed in sheep and goats (Prusiner, 1995). According to Cohen et al. (1994), prions cause a variety of degenerative neurologic diseases that can be infectious, inherited, or sporadic in origin. The cause of the sporadic forms is unknown; inherited forms are caused by up to twenty different mutations of the human PrP gene; and the infectious forms are transmitted through contact with or consumption of previously infected tissues (Prusiner, 1997).

PrPC is the normal, cellular prion protein, and it is converted into PrPSc (Prusiner, 1997). Mutations in the 102nd codon of this gene have been linked to neurodegeneration, which is the main, encompassing attribute of the prion diseases. Prusiner (1995) identified 15 amino acids at one end of the PrP protein. Using this knowledge, molecular probes were constructed and used to study the sequences of the normal verses the mutated form of the gene (Prusiner, 1995). Specifically, Prusiner discovered that the amino acid leucine is substituted by the amino acid proline (Prusiner, 1995). An incident of this type is commonly known as a point mutation. In the case of prion proteins, this mutation encodes additional copies of an octapeptide repeat toward the 5' end (Krakauer et al., 1997). The normal protein consists of mainly alpha helices with a spiral backbone, but the new, mutated prion protein is predominately formed by beta strands with a fully extended backbone (Prusiner, 1995). This alteration in tertiary structure provides evidence for post-translational modification of the protein. Observe the following illustration in order to see the tertiary structure of PrPSc:

FIGURE 1

It is essential to gain a more detailed understanding of the prion protein’s specific structure in order to comprehend kuru and other similar maladies.

Structure of a Prion Protein

The structure of a prion protein and its replication are fundamental to studying kuru. Although the precise details concerning the configuration of the prion were initially unclear, Prusiner was able to put forth three hypotheses. He claimed:

Hypotheses for the structure of the infectious prion particle included the following: (i) proteins surrounding a nucleic acid that encodes the proteins (a virus), (ii) proteins associated with a small polynucleotide, and (iii) proteins devoid of nucleic acid (Prusiner, 1991:1515).

Subsequent to the publication of this article, thousands of scientists tried to figure out the prion puzzle. According to Prusiner (1995), extracts from scrapie-infected brains were subjected to ultraviolet and ioninzing radiation (Prusiner, 1995). Such treatments usually destroy nucleic acids, but these tissues remained infectious (Prusiner, 1995). Prusiner (1995) concluded that the scrapie agent was indeed nucleotide-free, like a protein. This means that a prion does not contain DNA or RNA, which disproved Prusiner’s first hypothesis (that the prion could possibly be a virus). Furthermore, the prion was inactivated by extreme treatments that destroy or denature proteins, such as chaotropic ions or denaturing detergents (Cashman, 1997). After discovering these clues, scientists began to question the prion’s method of replication.

Cashman (1997) has suggested that the same nucleic acid and amino acid sequence gave rise to the two, different proteins. Further studies indicated the structural differences between the normal protein PrPC and the abnormal prion protein PrPSc. To summarize, the normal protein (PrPC) dissolves in nondenaturing detergents and breaks down easily with exposure to proteases, but PrPSc does not dissolve and is partially resistant to proteases (Prusiner, 1997). PrPSc can inhabit various acidic or basic environments, because it is stable between pH 2 and 10 and has survived two year immersions in formol saline (Mims and White, 1984). The last important feature to note with respect to the pathogenic prion particle is that the prion protein gene (PrP) in laboratory mice controlled the incubation time, neuropathology, and prion synthesis within the infected organism (Prusiner 1991).

Prion Replication

Scientists believe that the replication of a prion particle occurs almost exactly as the duplication of a virus. The mechanism of replication involves the synthesis of polypeptides in the absence of nucleic acid templates and the post-translational modifications of cellular proteins (Prusiner, 1991). A polypeptide is a chain of amino acids, and a nucleic acid template is a group of DNA or RNA molecules that carry information to direct cellular functions. For the prion, replication involves converting conventional proteins into prions. The resulting PrPSc is a four helix bundle protein with four regions of secondary structure, numbered H1 through H4 (Prusiner, 1997). Mestel (1996) explains that prions replicate by recruiting normal proteins to their cause, "flipping" them into a rogue prion-like shape that can go on to infect other cells and animals. This change initiates a chain reaction, and newly converted prions convert other proteins which they come into contact with on the interior of their respective cell membrane (Prusiner, 1995). In cell cultures, the conversion occurred inside neurons. The PrPSc accumulated in lysosomes and eventually filled the lysosomes until they exploded, releasing the prions to attack other cells (Prusiner, 1995). Future understanding of the operation of the PrP gene could possibly lead to a manipulation of these conditions in patients with a prion disease.

Development of Therapies

Currently, Prusiner (1995) believes that a more comprehensive understanding of the three-dimensional structure of the PrP protein will lead to the development of therapies. According to Prusiner (1995), experiments with laboratory mice were conducted. The PrP gene was targeted, and mice lacking the gene were created (Prusiner, 1995). In this case, the animals did not display any noticeable side effects or abnormalities (Prusiner, 1995). This is encouraging: if further studies show the PrP gene to be inessential, then physicians may be able to inject antigene therapies to patients with prion diseases in the future (Prusiner, 1995).

Recently, prion infections have been termed amyloidoses (Serpell et al., 1997). Serpell and colleagues state, "Amyloidoses are diseases...in which soluble proteins are deposited in a specific, highly stable, fibrillar form" (Serpell et al., 1997:871). Amyloid fibrils have three diagnostic characteristics: under the electron microscope, the fibrils are straight and unbranched with a smooth surface; amyloid fibrils can be stained with Congo Red and subsequently exhibit an apple-green birefringe; and they have a distinct X-ray defraction pattern, indicative of the beta sheets found in the PrPSc formation (Serpell et al., 1997). The following picture illustrates the hydrogen bonding patterns in beta-sheet structure:

FIGURE 2

Understanding the process of amyloid formation may aid in the development of therapies for such diseases.

Since the 1950s, scientists have worked on the prion puzzle. Microbiologists and epidemiologists have been confused by the prions. Advancements have been made, especially in the 1990s. This can be evidenced by Prusiner's reception of the Nobel Prize in 1997. However, it has still been difficult to detect prion infection, track its transmission, and type the different strains (Cashman, 1997). The Fore experienced a long struggle with kuru, which serves as a poignant example.

Conclusion

Since the discovery of the kuru epidemic in New Guinea, a vast amount of knowledge has been gained concerning prion diseases. The specific dynamics of the kuru disease are important to realize in order to better understand all prion diseases. Scientists admit that there is still a lot of ground to cover in this area of research. Numerous questions have been answered, yet many puzzles still remain to be solved. A large amount of the work done in the name of understanding prion diseases was carried out by anthropologists in the field studying the Fore. Their contributions to this research have played an enormous role. Fortunately, kuru has disappeared in New Guinea, but many prion diseases remain that can attack humans and animals. Although the case may be closed for kuru, the other prion diseases must continue to be studied in the hopes of conquering these illnesses.

History of Prions

Cannibalistic rituals have taken place among the women and children of the Fore tribe of Papua New Guinea for hundreds of years. These cannibalistic ceremonies of devouring the brains and bodies of departed loved ones were once thought to give honor to the deceased, but instead they have caused the death of many women and children. The cause of death from these acts of cannibalism was a mystery until the 1950s, when Carleton Gajdusek studied the Fore tribe and the disease that became known as kuru.

Kuru attacks the dura mater of the brain by riveting it with tiny vacuoles, causing the infected person to deteriorate rapidly and die within three months. Gajdusek won the Nobel Laureate Prize for Medicine in 1976 for his discovery of kuru, known as the “slow virus.” Due to the eradication of cannibalism in Papua New Guinea, kuru has virtually disappeared.

However, a scientist named Stanley Prusiner discovered a link between kuru and several other deadly diseases such as Creutzfeldt-Jakob disease (CJD), scrapie and bovine spongiform encephalopathy (BSE). Prusiner discovered that a mutated prion was responsible for the transmissible spongiform encephalopathy diseases that virtually turned the dura mater of human and animal brains into a sponge-like substance full of tiny star-shaped crystallized holes.

In 1997, the discovery of mutated prions earned Prusiner the Nobel Prize for Medicine. Although there are several prion diseases, CJD and kuru are the only ones that affect humans.

Recently in the UK and Europe, 51 people died from a new variation of CJD. Unlike traditional CJD, this new variant named vCJD can appear at any age. VCJD has been linked to the same infectious prion strain as BSE or “mad cow disease.” Scientists previously thought that BSE would not affect humans, but these recent deaths prove that the infectious prion disease is capable of much more than expected. Experts have theorized that the cause of the vCJD illness was ingesting beef that was contaminated with BSE. The deaths in Europe have raised concerns about infectious prion diseases and their capability to jump species.

Transmissible Spongiform Encephalopathy (TSE)

The table below shows several different sources of prion-related diseases and the species they affect.

Species	Disease
Man, Apes	Kuru
Man, Apes	Creutzfeldt-Jakob Disease (CJD)
Sheep	Scrapie
Cattle	Bovine Spongiform Encephalopathy (BSE) “Mad Cow Disease”
Man	vCJD (same prion strain as “Mad Cow Disease”)

Humans are also susceptible to several prion diseases:
1. Acquired infection (diet and following medical procedures such as surgery, growth hormone injections, corneal transplants) i.e. infectious agent implicated.

They are still using "growth hormone" in cattle. That raises a flag, wouldn't you think?

Oh, and thanks for all that information. I appreciate it.

I raise cattle myself. (Well actually just starting. I've only got a small start of a herd at this time, but I'm expecting a few calves anytime now and my herd is growing however slowly ;) But my cows are eau natural, all the way. I only feed rolled corn and oats 3 weeks before I butcher, other wise it's grass, hay and well water).

I know slow growth hurts being competitive; but those hormones are not suppose to be in our food.

Did you see the thread I wrote yesterday?

http://www.freerepublic.com/focus/f-news/1573646/posts
How do you say No NAIS in Japanese?

I would NEVER use growth hormone.

I'll have to check that thread out. Didn't see it yesterday.

There was a cluster of people in NJ, who had all frequented a race track, either working there or just going to the races, about five people I think.

Mrs VS