Randy, don't call the healthy PrPC protein a prion, it's not a PRION!!
These genetically modified mice, are they the so-called "knock-out" mice developed that don't produce PrPC proteins, or, are they a new GM mouse that produces only a portion of the natural healthy PrPC protein?
(By the way, if these scientists can reproduce the portion of the prion which causes tranmission of disease, then don't you think they know what the make-up and ingredients are of these prions?? Where is their magic formula?)
Either way, they are not animals which "naturally exist". There usefullness in the lab, is very limited by this.
I imagine a chemical which works something like chemotherapy could be used on people to prevent the anchoring of the PrPC protein to a cell membrane, but, slow down here.
As Randy said, their is a genuine purpose for the healthy PrPC protein, and it has been decribed by Dr. David Brown and others. Brown found that without its natural function, certain enzymatic functions were decreased, and this could cause cell death. What kind of damaging effects would occur to the brain if it was starved of the natural function of the PrPC protein? The knock-out mice, didn't fair so well in the lab. Being alive doesn't mean they are healthy.
Some chemo's, like the one I had, caused the fastest growing cells in my body to be damaged/killed and this stopped the rapid growth of the cancer cells (hopefully). Most, but not all malignant cells grow faster than our other healthy cells. Hair falls out because it is one of the fastest growing proteins. Luckily, this damage was short term and my hair grew back, although it is now a salt and pepper color.
Could a therapy that blocked PrPSc attachment to cell membranes work? Interesting question, but, as Randy pointed out the damage to the cell occurs with the accummulation of the prion on the cell, basically suffocating it of its nutrients.
Accummulation of plaques which cannot cause transmission, verses plaques which do, is an extremely important field of research.
Working with genetically modified animals with the intent to adapt this to the cattle industry is horrific. I would be far more concerned about the effects of eating GM foods, than I am about eating prions.
I personally, would prefer to see the research which uses cattle and BSE. I am still confused why experimental transmission studies on mice continue to use the scrapie prion. Surely we can source enough BSE prion from the UK to use in these experiments. Do not scientists like Prusiner, Aguzzi, have formulas for reproducing BSE prions?
I think Randy's points are important and well-said:
If this information spent anywhere near the time on the bench of scientists around the world as the "find a cure studies" we would not have to waste near as much time finding the ultimate solution. The probelm is reader, all the folks looking for solutions have no proof of cause with which to begin their research.
Putting the cart before the horse = no profit, no gain.
This is what has plagued our medical system for years and years. If no one was sick, we wouldn't need doctors, nurses, drugs, hospitals etc.