Vol 1 - No.3

What the devil is a Prion?


Not being medical researchers, we wouldn’t normally write this article. But so many
of you have asked for a basic explanation of the “mad cow” problem, here’s our attempt
to explain it, along with some observations, predictions and recommendations. (Our planned newsletter on easy home gardening for safe food will follow in a later issue.)

In 1972, Dr. Stanley Prusiner was a resident in neurology at the University of California School of Medicine at San Francisco, across the Bay from us. He had lost a patient to Creutzfeldt-Jakob disease (CJD). In the process of investigating the disease, which is a slow developing, brain wasting illness, he found others in Europe who had made some independent and startlingly relevant discoveries about possible causes.


He set up a laboratory at the U.C.S.F. in 1974 to seriously investigate the brain wasting diseases like CJD, scrapie (a disease of sheep) and their causes. Ultimately he, his fellow U.C.S.F. researchers and others discovered proteins that had no DNA or RNA. They were deformed and unlike any proteins previously known in medicine. In 1984 they coined the name prion proteins for these newly tagged protein forms. (By 1997, Dr. Prusiner had become a Nobel Laureate in Medicine! Well done!)


These prions are grouped as TSEs (Transmissible Spongiform Encephalopathies), which include BSE, scrapie, (human variant) vCJD, etc. These create a CNS (central nervous system) disease or diseases, effected through brain perforate reduction, or “wasting.”


Prions differ radically from the viruses, bacteria, parasites and fungi with which we are all familiar. They cannot be detected before death nor destroyed by sterilization. They seem to settle into the lymphatic part of the circulatory system, tonsils, spleen, etc. and sit there almost benignly until they begin their assault on the brain.  Oddly enough, we are learning from prions which occur in yeasts. Prions are mutated forms of proteins which “fold” and, in yeasts, can occur as fibrous chains. Yes, prions are not limited to cattle, sheep, etc. They have probably been around a long time. But because we’ve had the offal of prion-diseased animals made into feed supplements and fed to other animals, fish and poultry around much of the world, TSEs are appearing medically everywhere. The writers seriously doubt that the U.S. and Canada are immune to this onslaught – merely that we don’t yet know it’s here! We’re honestly expecting it to be announced at any time now in animals somewhere in the U.S. or Canada. Prof. John Collinge of St. Mary’s Hospital in London places the incubation period for TSE-caused CNS diseases at between 10 and as much as 30 years to evidence themselves in an individual. This is an estimate based on the current British cases and would forecast that many thousands more cases would occur in the next 15 to 25 years in people already infected throughout an unknown portion of Europe. It is quite an insidious family of neurological diseases that is caused by these animal prions.


The USDA and FDA are starting to take extraordinary emergency steps to try to keep infected food animals and offal feed additives from entering the U.S., as is Canada’s Food Inspection Agency under the Health of Animals Act. However, if you’ve read the prior newsletters, you know neither of the U.S. agencies is properly funded or manned to realistically cope with these carcass and feed inspections and the NAFTA practically prohibits an effective border defense. Other WTO treaties may also block effective defensive actions. Again, we don’t know. Canada may soon have the same inspection problems if it doesn’t already. They all need help – there’s no one else out there in the front lines that can guard our health interests and this could become a critical and truly unsolvable crisis at any time! We have no information on any attempts by Mexico to treat this problem effectively. Doesn’t mean they aren’t; just that we don’t know for sure.


While these recently discovered (food) pathogens have apparently existed for many generations, the new free trade of slaughter-offal feed additives from diseased carcasses appears to have spread the prion plague rapidly and extensively to many parts of the world, much as air travel has spread other human plagues to places unfamiliar with and defenseless against such foreign diseases. Just consider West Nile Fever, AIDS, etc.


Here’s the drill. If we don’t get our inspection and containment capabilities up to par right now, we don’t stand a chance to cope with a verified outbreak. Look at Britain and the EU – they’re in an advanced state of panic. Not just fear – PANIC! They can’t cope! And we’re already hearing the first murmers of trouble here in New England and Canada!


Should you panic? Of course not; we don’t know enough yet to know whether or not to panic. But you’d certainly better take positive steps to know the pre-slaughter treatment of your family’s meats and fish. Ask, then ask again! DEMAND! Fish and animal farms which crowd our growing food “products” and feed them cooked offal, antibiotics, etc. create many problems for the consumer. Don’t go there – at least until you know for a fact that effective corrective action has been discovered and implemented. You and your children deserve that protection! And that brings up a related matter . . . . .


Last year, the U.S. government spent less than $20 million researching prions (as far as we can tell.) Research is where it’s at until we know enough to become among the “prion intelligent.” Your congresscritters are mandated by our Constitution to “promote the general welfare.” Well, there’s not much “welfare” more general than eating – we all do it. Here’s our strong suggestion to help our uninformed in Washington to honorably and faithfully acquit their oaths of office:




1. Appropriate an adequate amount of research money and place it in the right hands. AND DO IT NOW!  NO add-ons, amendments or mark ups – just clean legislation covering accountable research to discover how, where and why we have this critical danger in our food supply chain. After we get nature’s ground rules on prions, we can go for mitigation and maybe even the complete prevention of TSE-caused CNS diseases.


2. Close our borders to any imports which MIGHT be suspect. IMMEDIATELY “beef” up the USDA and FDA capabilities with emergency appropriations. It takes time to train new inspectors and orient food scientists and time may be the one thing we lack! Grab the talent needed wherever it’s found. We’re most probably entering a life and death crisis! WE MUST MOBILIZE AND APPLY THE NEEDED TALENT NOW!

In other words,

“Sound General Quarters – All Hands to Battle Stations! This is not a drill!”


3. Seek and find existing meat, fish and poultry stocks which might be contaminated with prions – the TSEs. Quarantine them for monitoring and where indicated, responsible investigative experimentation. Some of this may have been done – some, but likely not all that should be. Pay the farmers and ranchers immediately for the “withheld” stock. They need the cash flow and our support! After all, we all permitted this to happen!


4. Hold the fort until we can make sense of all these unknowns and take effective action.


As for right now? Practice safe eating. You can’t put a condom on a prion!  K


The also-alarmed Embarcadero Publishing Staff


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