Bitterroot Star - March 24, 2021

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Covering the Bitterroot Valley – “Where Montana Begins!” – Established 1985 – Locally Owned & Independent

’ ! l a c o L t a t s e B ‘The

Wednesday, March 24, 2021

www.bitterrootstar.com

Volume XXXVI, Number 36

Scientists seek improved quality of life One of the horse scenes that Jane Lambert painted on tiles that now grace the wall of her kitchen. Victoria Howell photo.

Agriculture and craftsmanship lands valley local on French TV by Victoria Howell Jane Lambert of Stevensville has always loved horses. And she’s always loved art, especially the work of famous Western artist Charlie Russell. But she never could have imagined that those two loves would wind up with her being featured on a French television show. Jane, 77, was raised on the place her family homesteaded in Lake County, California in 1850. She got her first horse at the age of 6. She’s skilled in all the aspects of ranching life, and is also an accomplished artist, starting with leatherwork at age 7, and adding stained glass, jewelry making, sculpture, cinch weaving, basketry and china painting, among others. 1981 found her as a single mom teaching home economics and vocational-agricultural education. She was wanting to move her daughter out of California which was becoming rampant with drugs. She visited friends in Victor, Montana and fell in love with the Bitterroot. So, Jane took an early retirement and “took a chance on an eagle wing.” Locating in the Bitterroot Valley, Jane had trouble finding a teaching job. Her first job was picking apples for Swanson’s Orchards in Corvallis. From picking apples she moved on to cider making, cleaning, driving tractor, feeding cattle. “My ag background kept me alive,” she says, but barely. She milked cows for a while before landing a job as Vo-

Ag teacher at Big Sky High School for a year, filling in for a teacher on leave. In 1982, she met her future husband, Eric, at a St. Mary’s Saddle Club luncheon. Their first date was a breakfast ride. Together they opened a business called The Feed Bag, selling livestock feed. After selling that business, she became the manager of Feed & Fuel, a local feed and supply store, becoming known locally for her unique window displays. But eventually her allergies got the better of her and she had to leave that job. She did what so many others do to make a living here; she became a real estate agent. One of her ads pictured her on her white mule with the caption, “I don’t just sit on my ass, I sell real estate too.” Jane and Eric had a ranch on Sunset Bench and it was while running the ranch that Jane turned to freelance writing, starting out with “poison pen letters to the editor.” (Jane laughed when reminded that when the Bitterroot Star began publishing in 1985, she sent the paper a copy of the front page with the dozens of mistakes marked in red.) Jane always enjoyed the art of Charlie Russell and kept some of it around. “His depictions always rang true to me,” says Jane. “You look at a Charlie Russell picture and everything about it is right.” And, she notes, so much of what happens on ranches is still being done the same way. See Lambert, page 6

Author Jane Lambert in the “Charlie Russell corner” of her Stevensville house, holding two of the books she wrote. Victoria Howell photo.

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Dr. Cathryn Haigh at Rocky Mountain Laboratory holds aloft a flask containing human cerebral organoids, a kind of ‘mini-brain’ that scientists are using to test potential treatments for brain destroying neurological diseases like Creutzfeldt-Jakob disease. Photo by Michael Howell

NIH scientists use human cerebral organoid to test drug for deadly brain disease by Michael Howell It was about two years ago that National Institutes of Allergies and Infectious Diseases researcher Dr. Cathryn Haigh at Rocky Mountain Laboratory in Hamilton held aloft a flask containing cerebral organoids, a laboratory produced “minibrain,” that she and her team of scientists used to demonstrate that a prion disease, in this case sporadic Creutzfeldt-Jakob disease (CJD), could be transmitted to human brain cells and studied in vitro in the laboratory. CJD is a fatal neurodegenerative brain disease of humans believed to be caused by infectious prion protein. It can arise spontaneously, result from a hereditary mutation within the prion gene, or arise due to infection, for example, from eating contaminated meat products. A notable example of this occurred in the United Kingdom in the mid-1990s following an outbreak of bovine spongiform encephalopathy in cattle. There are no preventive or therapeutic treatments for CJD. Human cerebral organoids are small balls of human brain cells ranging in size from a poppy seed to a small pea. Their organization, structure, and electrical signaling are similar to brain tissue. Because these cerebral organoids can survive in a controlled environment for months, nervous system diseases, like CJD, can be studied over time. The lack of a completely human CJD model has been a considerable barrier hindering the discovery of potential therapies. Studies in mice have failed to identify treatments that were then effective when tried in patients. By using human cerebral organoid CJD they are getting one step closer to identifying potential treatments for the disease. Haigh and her team didn’t invent

the technique of growing cultured brain tissue. Their contribution was in creating this prion infection model by demonstrating that a prion disease can be transmitted to human brain cells. One part of the process that Haigh appreciates is that you don’t need to start with any human brain cells. You make them. All the participating patient has to contribute is a small sample of skin. These skin cells are then “genetically engineered,” reducing them to stem cells and then stimulating the development of brain cells out of that stem component by administering certain hormones. In their latest article published in Scientific Reports [Human cerebral organoids as a therapeutic drug screening model for Creutzfeldt-Jakob Disease. Scientific Reports DOI: 10.1038/s41598-021-84689-6 (2021)], authors B. Groveman and NC Ferreira et al, describe the laboratory work which involved testing pentosan polysulfate (PPS) to determine its potential preventive and therapeutic benefits. PPS is a benchmark anti-prion compound in laboratory experiments. Haigh calls it the “gold standard.” It is rarely used clinically because it requires direct administration into the brain. Haigh said that, despite its effectiveness at stopping the disease, it would never be a proper therapy because it has to be delivered by pumping directly into the brain and, while it may extend a patient’s life, PPS has not been shown to improve quality of life. “We don’t just want to stop the disease,” said Haigh, “we want recovery. We want to help the neurons recover normal functioning.” By using the anti-prion properties of PPS with the new human organoid CJD model, the researchers were able to assess the value of this model system for drug discovery. According to Haigh, the tests showed that the

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human organoid model can be used to screen compounds that may be useful for preventive treatment. According to Haigh, two different methods were used in the study. In the first, the brain organoids were infected but at the same time given the PPS treatment to see if it would work as a prophylactic. The second method involved testing the treatment after the infection had fully set in to see if the treatment had therapeutic value. “Both paradigms showed a really significant reduction in infection,” said Haigh. Such treatment could be used for people carrying genetic mutations that cause the disease, but who have not yet developed symptoms, or for people who may have been exposed to infectious prion proteins that might cause CJD. The model further proved useful for screening drugs against established CJD after a patient is diagnosed and starts showing symptoms of disease. Haigh said scientists around the world are working to expand the organoid model for screening larger numbers of novel drug candidates. Their goal is to find treatment options for people who are susceptible to CJD because of their genetics or who accidentally are exposed, as well as for those who develop sporadic disease. They are optimistic that with their fully human model of disease, they can now identify compounds with promise for benefitting patients with CJD. Cerebral organoids are also being used as models to study Zika virus infection, Alzheimer’s disease, and Down syndrome. According to Haigh, finding a treatment that can cross the blood/ brain barrier and diffuse into the brain without having to be pumped directly into the brain is critical to developing a successful therapeutic treatment for the disease. “There is so much going on and each little step takes us closer to the summit,” said Haigh.

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