Sheep may be key in treatment for Huntington’s Disease

Carrier animals grown in producer flocks

Connie Sieh Groop
Special to the Farm Forum

A 2014 meeting in Aberdeen shared information about a nervous system disorder in sheep that might someday help people who suffer from Huntington’s disease or Parkinson’s disease.

Pete Kronberg of Forbes was one who was excited to learn he could breed animals that would produce lambs that might provide the needed tissue to fight the disease.

“It’s such a worthwhile cause,” Pete said. He purchased bucks from Larry and Sue Holler to help with the process. “I’d be hard-pressed to find something else as worthwhile. As long as Larry and Sue Holler remain involved, we’ll continue to raise these sheep. We have about 100 carrier ewes determined by DNA samples.”

Larry and Sue Holler focused on this novel research for about 30 years at their farm at White, SD. They created GlycoScience Research which works toward clinical trials with GM1 ganglioside for Huntingtons’s Disease. Funding from The Bev Hartig Huntington’s Disease Foundation allowed them to set up a lab at the Brookings Research Park and collaborate with scientists at the USD Graduate Education and Applied Research Center in Sioux Falls. Additional funding comes through sheep industry grants and The Shepherd’s Gift: GM1 for HD, a non-profit founded by HD families.

HD has been called the cruelest disease known to man and described as Parkinson’s, ALS and Alzheimer’s all rolled into one disease. GM1 ganglioside shows promise for halting the progression of HD and possibly reversing symptoms. GM1 is a natural molecule found in brain cells of all mammals, as a treatment it protects and regenerates neurons. A special genetic line of sheep, which have GM1 gangliosidosis, accumulate GM1 in levels 40 times normal and can be the replacement source of this essential protection needed for the brain cells of these patients.

In December, Larry turned 63. Surviving a year when his heart was rebuilt and overcame Methicillin-resistant Staphylococcus aureus (MRSA), he is back to work at the South Dakota State University Animal Diagnostic lab. He works as a researcher and veterinarian while his wife Sue raises animals on their farm.

This week, Sue, assisted by Hannah Hart and Sampson Asare at the GSR lab, submitted a grant request to the National Center for Advancement of Translational Science, part of the National Institutes of Health. The NIH is responsible for biomedical and public health research. The grant funding would continue the research the Hollers see as vital.

The grant states: “The possible applications for GM1 as a therapeutic across diseases cannot be understated, and no other product sold or manufactured is equal to the potential benefits of ovine GM1. GSR has the only source of GM1 in the world that is traceable and verified. Far too many projects, clinical trials, and people suffering from diseases have had progress stifled, simply because of a lack of GM1 supply and traceability. Millions of patients could benefit from GM1 treatment.”

In seeking funding, Larry said, it’s hard to get people at the Food and Drug Administration and the USDA to understand how agriculture fits into providing a solution to fight diseases.

Larry emphasized, “We are emotionally involved, it’s not just a project for us. We met many families who are waiting for a medication that could fight this disease. We have good friends who have died while waiting. We want to succeed.”

Processing of affected animals takes place at the Holler farm. They euthanize the animals, package and label the tissue. Freezers of the tissue are ready when needed for the research.

“We can only do so much with the funds we receive,” Larry said. “We appreciate the support but don’t have enough yet for a clinical trial. There are many challenges but we are optimistic that we will get funding to continue. Currently, a lot of research dollars are directed towards the coronavirus as it should be.”

The network continues to be ready. When needed, those like Pete, will raise needed animals with carrier genetics through selective breeding.

Through the process, those who raised affected lambs received a premium price which had a tremendous impact on supporting sheep operations. Susan and their son processed about 60 lambs this year, but they will discontinue the process because of Larry’s health and stalling of the research. “We could only use so many. The good news is that we have a lot of tissue in our freezer if the funding for the research comes through for the trials.”

For now, it’s a waiting game. “It’s no cost to me to have them in the herd,” Pete said. “For now, we raise the lambs and put a notch in their ear. When it comes time, we send them down the road like our other lambs. But the flock could start producing affected animals through breeding when needed when drug trials begin. As long as the Hollers are looking at this process, we’ll do what we can.”

Larry sums up his feelings: “We love raising the sheep, and the research has become critical to what we do in terms of the hope that might bring to those affected by HD.” Find out more at www.glycoscienceresearch.com/ or www.theshepherdsgift.org