According to a new French study, proteins specific to Alzheimer’s disease behave like pathogenic prions, transmitting their abnormal form to healthy prion proteins. In the case of cattle prions are also responsible for the transmission of Mad Cow disease.
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A Woman With Alzheimer
Alzheimer’s disease is the leading cause of dementia in the world. It affects 8 million new people every year. From a clinical point of view, it progressively affects the patient’s cognitive functions (memory, language, reasoning, learning, problem solving, decision-making, perception, attention…) leading to a loss of autonomy. From a physiological point of view, it has been known for thirty years that two proteins play a central role in their formation: the beta-amyloid protein and the tau protein. According to a French study published in Acta Neuropathologica Communications, these proteins behave as pathogenic prions, transmitting their abnormal form to healthy prion proteins and thus multiplying the degeneration of neurons in the brain.
To carry out their study, researchers at the François Jacob Institute of Biology (France) examined samples of patients who died of Alzheimer’s disease. By inoculating extracts in healthy animals, they observed that this eventually led to memory and speech disorders, loss of neurons, and a reduction in brain size.
They also observed the appearance of lesions characteristic of Alzheimer’s disease: amyloidosis and tauopathy. Thus, transmission of disease damage suggests that β-amyloid and Tau proteins behave as pathogenic prions, the researchers observed.
First such experimental demonstration
On its website, Inserm explains that “prion diseases are rare diseases characterized by a degeneration of the central nervous system and the formation of aggregates of a specific protein”. They are due to the accumulation in the brain of a normal but malformed protein, the prion protein. These diseases are characterized by a rapid and fatal course and absence of treatment. The best known is Creutzfeldt-Jakob disease”.
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Although this study is not the first to study the “prion hypothesis” in Alzheimer’s disease (a theory already advanced for other degenerative diseases such as Parkinson’s), it is the first experimental demonstration in which clinical signs associated with a neurodegenerative process were inoculated through the sample from one brain to another.
“Our results indicate that inoculation of brain homogenate of Alzheimer’s disease induces encephalopathy characterized by neuronal loss, progressive atrophy, changes in neuronal activity and cognitive deficits, as well as rare deposits of β-amyloid and tau. The clinical signs are explained by neuronal loss, brain atrophy and neuronal network dysfunction. Tau lesions may be a strong, but not the only, decisive induction of neuronal loss and clinical outcomes. Further studies are needed to assess the nature of the relationships between the different lesions induced by the inoculation of the Alzheimer’s gene and to evaluate the mechanisms leading to encephalopathy induced by these inoculations,” the researchers conclude.
A disease that is spreading
This discovery reinforces the Alzheimer’s prion hypothesis, paving the way for the exploration of new mechanisms to understand neuronal and memory loss. In the US, Alzheimer’s disease affects about 5.8 million people and more than 50 million worldwide.
References
- https://actaneurocomms.biomedcentral.com/articles/10.1186/s40478-017-0488-7
- https://presse.inserm.fr/en/a-new-therapeutic-strategy-to-combat-prion-and-alzheimers-diseases/9191/
Ms. Tagani
Could we be wrong about what causes dreaded chronic wasting disease in deer?
As the scourge of chronic wasting disease spreads among deer in Pennsylvania, most recently in Lancaster County, a medical researcher claims the very source of the disease has been misdiagnosed all along.
The disease threatens the $1.8-billion deer-hunting industry in Pennsylvania, as well as the state’s popular elk herd. There’s also a popular cottage industry in Lancaster County, where there are more than 70 deer farms.
The disease was first discovered in wild deer in the United States in 1981. The first infected deer found in Pennsylvania — at a deer farm — was in Adams County in 2012. Wild deer were discovered later the same year in Blair and Bedford counties.
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As the result of three deer found with the disease from a captive deer farm near Denver, a quarantine zone has been created in parts of northern Lancaster County, Berks and Lebanon.
Within the zone, hunters were required to deposit the heads of the deer they killed this past season for testing to see if the disease had spread into wild deer. Some 345 deer were collected. Most have been tested and so far no infected deer have been found, according to the Pennsylvania Game Commission.
In the Capitol Rotunda earlier this week, members of one of Pennsylvania’s largest hunting groups gathered to pledge money to help the iconoclastic research of Dr. Frank Bastian, a neuropathologist and animal scientist with Louisiana State University who claims to have discovered the real cause of chronic wasting disease.
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Bastian maintains wildlife managers trying to avert the crisis are barking up the wrong tree. He’s been preaching that for the past 12 years.
The accepted theory of every wildlife agency in the country, as well as the U.S. Centers for Disease Control and Prevention and the U.S. Fish and Wildlife Service, is that malformed proteins produce particles called prions that cause brain diseases such as chronic wasting disease, mad cow disease and sheep scrapie. Neurologist Dr. Stanley Prusiner won a Nobel Prize for this theory in 1997.
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Dr. Frank Bastian of LSU, who says state wildlife managers don’t know the real source of chronic wasting disease in deer.
LSU
But the prion explanation is simply wrong as a cause for chronic wasting disease, Bastian and Unified Sportsmen of Pennsylvania insist.
The source of the disease that kills deer without fail is not prions but a super-tiny bacterium, according to Bastian. The prion is only a secondary bacterial byproduct and a marker for deer with chronic wasting disease, he says.
What of it?
Well, everything. Bastian says that now that he has successfully grown the offending bacteria in a lab, he and other researchers can now develop a vaccine in the form of an antibiotic to treat, cure and prevent the disease in both captive deer and deer in the wild.
And, he hopes to develop a test kit that hunters can take into the woods and test deer they shoot to see if they are infected with the disease and safe to eat or not. Pennsylvania hunters may be the first to test the kits.
U.S. CENTERS FOR DISEASE CONTROL AND PREVENTION
Further research, if supported, could pave the way for the dreaded disease’s eradication in time and save deer hunting in Pennsylvania, according to Unified Sportsmen of Pennsylvania. The group hails the discovery as “breakthrough” and a “game changer.”
“We need answers now. Time is running out,” says Stephen Mohr of Bainbridge, chairman of the Unified Sportsmen of Pennsylvania board.
“Once CWD hits the Pennsylvania elk herd, it is over. They will be totally gone in 10 years or less.”
The rally prompted a quick retort from the Game Commission and the state Department of Agriculture, which are sticking to the prion theory The two agencies said they wanted “to make it clear that decades of research have provided abundant evidence that prions, or misfolded proteins, are the infectious agent of CWD.”
The Game Commission is well aware of Bastian’s research, says Courtney Colley, the agency’s chronic wasting disease communications specialist.
“One of the first things to point out is that the Game Commission would welcome any successful effort by any researcher that will provide a test for CWD,” she said. If he could develop a vaccine, we would love it because it would give us something to work with.”
But, she continued, “the one thing about Dr. Bastian’s research is that no one has been able to replicate it. No wildlife agency accepts the bacteria theory.
“As a state wildlife agency that manages wildlife in Pennsylvania, it would really be irresponsible of us not to follow the most widely accepted scientific research which is that prions are the cause of chronic wasting disease.”
Bastian’s 2017 research in which he reported his ability to isolate the bacteria from chronic wasting disease-infected deer was published in the Journal of Neuropathology and Experimental Neurology.
His findings have convinced Dr. Laura Manuelidis, a professor of neuropathology at Yale Medical School.
That prompted Deer and Deer Hunting magazine to editorialize, “Given how little is still known about the dreaded disease, and the fact that state wildlife agencies have been unable to stop it from spreading, it’s high time all scientists look closely at the entire body of CWD research and follow the path that science intended.”
If the truth is still out there, let’s hope the scientific process prevails before it’s too late for Pennsylvania’s revered whitetail deer and elk.