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What Are Prions - How
New Are They ?

From Ted Twietmeyer
tedtw@frontiernet.net
10-2-4
 
PRION BACKGROUND - Some information you may not yet know about
 
The prion diseases are a large group of related neurodegenerative conditions, which affect both animals and humans. Included are Creutzfeldt-Jakob disease (CJD) and Gerstmann-Sträussler-Scheinker (GSS) in humans; bovine spongiform encephalopathy (BSE), or mad cow disease, in cattle; chronic wasting disease (CWD) in mule deer and elk; and scrapie in sheep. These diseases all have long incubation periods but are typically rapidly progressive once clinical symptoms begin. All prion diseases are fatal, with no effective form of treatment currently; however, increased understanding of their pathogenesis has recently led to the promise of effective therapeutic interventions in the near future. [1]
 
There is also a variant CJD which has different pathological effects as explained in [2].
 
It is also noteworthy that Prion size was identified back in 1971. Structures have both straight shapes and spirals. In the photo below, a human strain is compared to hamster prion:
 
 
 
Image credit:
http://www.mad-cow.org/prion_structure_folder/human_mutsRIB_pnas.jpeg
 
 
 
FREQUENCY OF INFECTIONS (number of cases per million people):
 
 
* In the US: The most common prion disease is CJD, with a uniform incidence of approximately 1 case per million population both in the United States and internationally. Familial forms of prion diseases, such as GSS and fatal familial insomnia (FFI), are much more rare. About 10% of cases of CJD are familial, with an autosomal dominant pattern of inheritance linked to mutations in the PRNP gene.
 
* INTERNATIONALLY:
So far, over 100 cases of NiCad have been reported in the United Kingdom. Whether these patients represent the beginning of a growing epidemic (such as that which occurred with BSE) or whether the number of cases will remain relatively low remains unclear. For the past 2 years, incidence of nvCJD in the United Kingdom has remained stable, with 27 cases reported in 2000 and 20 cases reported in 2001. Within the first 6 months of 2002, 22 new cases have been recorded.
 
Two populations are affected by CJD: Libyan-born Israelis and some populations in restricted areas of Slovakia where the incidence of CJD is 60-100 times greater than expected. These clusters were postulated to be related to dietary exposure of the scrapie agent; however, this was not supported by case-controlled studies. These local high rates of CJD are linked to a high prevalence of codon 200 mutations in the PRNP gene. The human PrP gene (PRNP) is found on chromosome 20.
 
* The mean duration of sporadic CJD is 8 months.
 
* nvCJD has a slightly longer course, with a mean duration of 16 months.
 
* Familial CJD has a mean duration of 26 months, while GSS has the longest course, about 60 months.
 
RACE:
 
Sporadic CJD occurs throughout the world in people of all races and typically has similar features.
 
* Some familial forms of prion disease, such as familial CJD, can have distinct features in an ethnic group. For example, familial CJD in the Libyan Jewish population associated with a codon 200 mutation has features of a peripheral neuropathy in addition to the more typical manifestations of CJD (Meiner, 1992; Neufeld, 1992).
 
* nvCJD has been limited to Europe, with almost all cases occurring in the United Kingdom.
 
SEX:
 
No sex preponderance is known in prion diseases, with some rare exceptions. For example, women had a greater tendency than men to develop kuru because it was part of the ritual cannibalism for women to eat the brains (and neural tissue has the highest dose of PrPSc).
 
AGE:
 
* The mean age of onset of sporadic CJD is 62 years. The incidence of sporadic CJD is about 1 case per million population; however, among individuals aged 60-74 years, the incidence is 5 cases per million population (Holman, 1996). The age range can be broad; cases have been reported in people as young as 17 years and as old as 83 years (Masters, 1979; Cathala, 1987).
 
* nvCJD occurs in younger patients, with a mean age of onset of 29 years.
 
* Familial CJD, GSS, and FFI have mean ages of onset ranging from 45-49 years. [1]
 
 
WHY PRION PROTEINS ARE DIFFERENT FROM ORDINARY PROTEIN
A Prion is a rod-like protein that grows more in length than in diameter, penetrating and destroying brain tissue. Normal proteins fold, while prions do not. The mechanism is not yet well understood in the science community. Considerable research into prion has been taking place at various institutions in the world. When Prisons infect humans, it is called Creutzfeldt-Jakob Disease (pronounced "Crutz-felt Yah-cub", or known as simply as CJD.) There is strong evidence today that at least one strain of prions which infects cows, crosses the blood-brain barrier in humans by eating infected meat.
 
MORE VIRULENT THAN VIRUSES
People have survived some of the deadliest, fastest acting pathogens on earth such as Ebola which can kill in just 3 days. Although not considered 100% fatal as the movies suggest survival is rare, however. prions can withstand the high vacuum of space, standard sterilization techniques and temperatures in excess of 1,000 degrees. CJD is considered always to be 100% fatal. Recent news described the spread of the disease in Florida via medical instruments. These instruments were used in operating rooms on the surgery of 500 patients. Consider that this is just ONE known case voluntarily made public. Statistics would dictate that this terrible "accident" will already have happened across the world, in perhaps or even thousands of times in many hospitals.
The problem is most likely worse in third world countries, where disposable medical instruments may be rare or non-existent.
 
The Florida incident raises many troubling questions such as:
 
1 .Why haven't thousands or millions of people died already as a result of infected instruments and other causes ? Prion infections are considered 100% fatal.
 
2. Could a Prion epidemic be successfully covered up ? There have a few families of CJD patients, who were told by the hospital to keep quiet about the loss of a loved one. But when the number of CJD victims reaches thousands, even this form of damage control will not work.
 
3. The existence of prions has been known about for many years, and yet surgery continues with ineffective instrument sterilization. And little is said about raising the Standard of Care that doctors and hospitals follow to lower the risk of lawsuits. Why are hospitals not yet using ALL disposable instruments that contact bodily fluids, especially those instruments used for brain surgery ? One notices the safety glasses, face shields and other protection doctors and nurses now use around patients. Why are patients also not protected so carefully ?
 
4. In the UK it was found that prions are also present in the tears of infected patients. Some optical instruments, such as a physical contact-type Tonometer for measuring glaucoma, can transmit the disease from one patient to another. The doctor or office staff must disinfect the end of the Tonometer after each patient. But a survey showed this does not happen with regularity. And disinfect it with WHAT ? Is there something that is proven to work ? More advanced instruments used in the United States use a puff of filtered air to measure eye pressure, without any physical contact. It's amazing that physical contact Tonometers are still used. At the very least, a sterile throw-away plastic cover on the device can stop the spread of prions (CJD) and other eye diseases as well.
 
4. Do workers in meat and food processing factories have a higher incidence of this disease than non-workers ? Does anyone know ?
 
TRANSMISSION PATHS TO HUMANS
We must ask this question - have they always been here and just now being recognized ? This seems very likely.
 
All of us can be exposed to prions through various carriers such as:
 
* Medical and dental instruments - The current Standard of Care does not require disposable medical instruments for ALL
invasive medical procedures. You can be sure the penny-pinching medical community will not do this until ordered to do so.
This may or may not come in time.
 
* Cat and dog food, commonly made from road kill and sick animals, is only heated to about 250 degrees to process it.
 
* Meat and other food made from cows that may be infected, and were not detected (or looked for) in processing.
 
* Lipstick, made from the brains of slaughtered cattle. Since it is a cosmetic, this source of infection was never regulated until
recently. It may also be in other cosmetics as yet unrevealed.
 
* Contact with pets that eat infected pet food, but this is vector not yet proven.
 
There are other forms of transmission not covered above, but these are enough.
 
CRAZED WILDLIFE - RABIES OR MAD COW ?
 
Ever see a mad deer or other animal struggle to stand, or intentionally walk out on weak ice on a lake in early spring ? I've witnessed this happen - after rescuers risk their lives to release the animal from a certain deep freeze death, incredibly the animal immediately runs back out on the same ice again. Clearly something is wrong. One may not readily know whether the animal is in the last stages of rabies or has prion disease, since both diseases attack the brain. Only by removing the dead animal's head and sending it to a pathology lab can we know what the affliction is. Many police departments have rabies kits, an unwanted duty described to me by a local state police officer: The sick animal is shot and the officer puts on rubber gloves. He then decapitates the animal and places the head into a sealed plastic bag, along with the gloves. The remains of the animal are buried, and the bag is labeled, packed in ice and air shipped to a testing lab. He noted it is a most unpleasant duty and he avoids it any cost. Not to mention the paperwork it requires. How many mad cow type cases have been ignored over the years by less than publicly minded law enforcement officers ? That number is probably higher than we think.
 
HAVE PRIONS ALWAYS BEEN HERE ?
The current increase of these cases could be attributed to perhaps a new awareness in the medical community, or, the disease was not here before. We do know that prions were measured and detected as far back as 33 years ago. No one seems to be exploring the idea that we have been made susceptible to this horrific disease - one that has ALWAYS been here. Legionaires disease is a good example of this - caused by a bacterium present everywhere in ordinary earth. Yet until some 60 Legionaires died at a hotel back in the seventies no one knew it existed, hence the name. The same is true for cancer - how many millennia passed while humankind has been on the earth, before uncontrolled cell growth was called cancer ?
 
My theory (which I'm sure others may share) is that prions have always been here on earth. In some unexplained way, humans and animals may now be more susceptible to this pathogen than ever before. This susceptibility is indicated by changes in chromosome 20 (from research stated in the above extract.) Could it be there is something in our environment that triggers susceptibility ? Perhaps certain microwave RF frequencies such as those used by cell phones, may cause a protein mutation in those individuals who are already susceptible. Few people realize that a cell tower, with a typical power level of 100 WATTS per ANTENNA, with (four) three antenna arrays for a total of 12 antennas, RADIATES ALMOST DOUBLE THE POWER OF A TYPICAL 700 WATT MICROWAVE OVEN. Perhaps Prion susceptibility may be a chemical effect, caused by a common food additive.
 
Controlled scientific tests can determine what stimulates Prion growth. Rodents and primates will probably need to be sacrificed to accomplish this as a matter of performing the science needed to develop some conclusions. This won't make animal rights activists happy to read this. But these are the same people who, when they are ill and need an operation to save their life, forget all about animals. They don't want to die, and on that day no matter how many animals were sacrificed, it will all be irrelevant to them. Animal research isn't cheap- an adult Rhesus monkey today actually costs a researcher about $30,000.00. Why do they cost so much ? Because after birth, it takes months of very carefully feeding a monkey in an ultra-clean environment to raise it to the required maturity. An adult monkey has the mentality of about a four year old child. Funding such tests is another matter altogether, especially if the cause of prion growth implicates a billion dollar industry such as telephony. One can be assured that the telephony industry won't accept such results silently.
 
CJD type pathogens probably have always been here on Earth, but are now being stimulated into a growth mode. Other incurable diseases are quite useful for population control. For example, mycoplasmas, not HIV are the cause AIDS. According to NIH Report #9, dated 1969 AIDS it is not HIV as most think. HIV is an opportunistic infection and is the disease doctors are told to look for. This is just another example of an outdated Standard of Care. [3] By the time HIV is detected, a patient is already mycoplasma infected. AIDS, prions and other pathogens assist in very effective and almost unstoppable population control.
 
The reader will notice the terms "CJD" and nvCJD" used in this article. Please read the reference material given below to better understand the difference in the two types of prion infections.
 
Ted Twietmeyer is founder of data4science.net, which looks at scientific phenomena from a different perspective. The website also actively solicits volunteers to submit observations from around the world about various phenomena at http://www.data4science.net
 
REFERENCES
[1] Science on prions at http://www.emedicine.com/neuro/topic662.htm
 
[2] Variant CJD described http://www.bchealthguide.org/healthfiles/hfile55b.stm
 
[3] Professor Don Scott and mycoplasmas - http://www.nexusmagazine.com/articles/mycoplasma.html
 
 

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