A $454,967 federal research award will help associate biology professor Andrew Voss and his Wright State University students investigate Huntington’s disease, a fatal genetic disorder that causes the progressive breakdown of nerve cells in the brain and possibly skeletal muscle.
Huntington’s disease, which has no cure, deteriorates a person’s physical and mental abilities during their prime working years. Today, there are about 30,000 symptomatic Americans and more than 200,000 at risk of inheriting the disease.
The three-year research award from the National Institutes of Health/National Institute of Neurological Disorders and Stroke is aimed at determining if there is a primary disease of the muscle in Huntington’s disease.
“We did find defects in the muscle of Huntington’s disease mice,” said Voss. “But the question is how much of that is due to a nerve degeneration versus how much of it is muscle itself? And do the muscle defects also feedback to the nervous system?”
Using the same tools that are used in gene therapy, Voss’ students are inserting the mutant Huntington gene into skeletal muscle to determine the role of the mutant Huntington gene in skeletal muscle and how muscle signals back to nerves.
The outcome of the research holds out the promise of paving the way for treatment of Huntington’s. The other benefit may be an increased ability to assess and track the progression of the disease.
Symptoms of Huntington’s usually appear between the ages of 30 to 50 and worsen over a 10-to-25-year period. Ultimately, the weakened individual succumbs to pneumonia, heart failure or other complications.
Voss’ journey to Huntington’s research was a winding road. He grew up in rural Minnesota and moved to Sacramento, California, when he was in junior high school. After graduating high school, he enrolled at the University of California-Davis, got interested in biology and then biochemistry.
“The longer I was in biology the more I began to blend it with some of the physical sciences,” he said. “You have biology with lots of variables and then you get the physical side, which gives you more of a concrete, repeatable picture.”
After earning his bachelor’s degree in biochemistry in 1996, Voss taught chemistry and basic science at Hiram W. Johnson High School, an inner-city school in Sacramento.
“It gave me a good appreciation for teaching and to see what kids in the inner city go through,” he said. “It was all about my interaction with the kids, which was great. But I wanted to do more actual science.”
So in 2004, Voss returned to UC-Davis, earning his Ph.D. in pharmacology and toxicology. He then did postdoctoral work at UCLA’s medical school, where his interest shifted to physiology.
“What I really liked was some of the electrophysiology — the electrical signals in a nerve and muscle,” he said. “I really liked the application of that technique.
In 2009, he landed a faculty position at California Polytechnic State University in Pomona teaching human physiology. While he was teaching a graduate class, one of the students selected a primary research paper that examined neuromuscular junctions in Huntington’s disease mice.
“I saw the data a little bit differently,” Voss recalled. “I hypothesized that there was actually a problem in the muscle, so I began conducting experiments with the mice and discovered that there was in fact a defect.”
The discovery led to a research paper on Huntington’s and publication in a prestigious academic journal.
Voss, who joined the faculty at Wright State in 2014, points out that the Huntington’s research is being done by his undergraduate and graduate students.
“It’s important for them to be able to do the experiments,” he said.