Scientists report progress in prion research

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Researchers at the University of Alberta’s Centre for Prions and Protein Folding Diseases report finding a protective mechanism in cells related to the slow onset of symptoms, which could lead to methods for preventing the onset of neurological diseases such as Creutzfeldt-Jakob disease (CJD) in humans and bovine spongiform encephalopathy (BSE) in cattle.

Their report, titled “Prion disease tempo determined by host-dependent substrate reduction,” is published in The Journal of Clinical Investigation.

The findings "present a breakthrough in understanding the secret life of prion molecules in the brain and may offer a new way to treat prion diseases," says David Westaway, PhD, a researcher on the project.  Quoted in an article in the Edmonton Sun, Westaway says "We think we have stumbled upon something unexpected, that the cells have a protective mechanism. And we are quite confident that finding is very solid, very robust, because we looked at six different types of prion disease and they all had the same effect."

Prion diseases such as CJD typically have long incubation periods, but can progress rapidly after the onset of symptoms, and there is no cure.

Using rodent models, the researchers found reductions in PrPC, the cellular precursor for the misfolded prion protein, during the incubation period. These reductions occur in a wide range of prion diseases, including sheep scrapie, human Creutzfeldt-Jakob disease, and cervid chronic wasting disease. They report this “downregulation” of PrPC is not discernible in animals with unusually short incubation periods but is evident in slowly evolving prion infections. “Our data reveal PrPC downregulation as a previously unappreciated element of disease pathogenesis that defines the extensive, presymptomatic period for many prion strains,” the researchers note in the report’s abstract.

The group plans to continue research into ways to augment the protective response to keep prion diseases from advancing beyond their pre-clinical phase.

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January, 21, 2014 at 11:36 AM

All good health starts with genuinely healthy soil; no viable shortcuts or replacements exist. Treatment of disease should always begin with finding and eliminating human errors that lead to deficiencies and disease, optimizing natural wellness to enhance safeguards like immune response. Within the next decades, hopefully, as science regains some humility, we will see more emphasis on wellness to prevent disease and less emphasis on treatment of degenerative illnesses that result from sources like inferior or incomplete nutrition, chemical interference and pollution. Much scientific research spends inordinate amounts of money trying to reinvent the "wheel of life" in ways that defy natural laws, like the law of return, for example. Bovine spongiform encephalopathy developed when human beings tried to short-cut the protein nutrition of their cattle, essentially turning them into cannibals by feeding them animal by-products. Ruminants are not designed to consume animal sources of protein. Degenerative disease comes from that kind of top-down nutritional manipulation, when human beings try to impose mainly profit-oriented human short-cuts on natural systems that have worked well for tens of thousands of years. Earth-up nutrition has always safeguarded the organisms that naturally get their nutrition from healthy soils. Conjugated linoleic acid, for example, available from the meat and milk of ruminant animals that graze healthy grass grown on healthy soils, is among the best nutritional safeguards available for human health. With adequate amounts of such foods in our diets, we have the greatest natural protection available against cancer, diabetes, heart disease, and a host of other degenerative diseases like CJD.

January, 25, 2014 at 07:18 AM

What an iconic picture you paint. Naive, but a nice thought. All soils everywhere are lacking in complete nutrition or often have an overabundance of a few nutrients that cause concerns. Although it varies from one soil type and geographic area to another - even locally. In addition plants don't necessarily take up nutrients completely or efficiently, so blaming most diseases on a lack of "healthy soil" is misleading. What is your definition of "healthy soil"? Please explain how neurologic disease CWD in deer is caused by feeding infected animal protein? How about scrapie? CJD? I guess no BSE existed before meat and bone meal was fed? BSE developed when humans tried to short cut protein nutrition of cattle by turning them into cannibals? Your statements are sensational, but not factual. Grazing cattle on high quality pasture on excellent soild are still lacking in some nutrients and consuming excesses of others. Even on native lands in all regions of the world where humans have never "managed" the lands. Even before humans walked the earth But, of course, the radicals will paint humans as the cause of all bad...

SD  |  January, 25, 2014 at 05:58 PM

Haven't we heard for many years how ancient people burned bones of animals for the minerals for either their farming or their somewhat domesticated animals? It seems reasonable to think wild animals spend some time gnawing on decomposing bones of other animals to gain nutrients their bodies need. Probably as long as animals have existed.

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