Neurodegenerative disorders like Huntington’s and Alzheimer’s share a common underlying feature–the abnormal aggregation of proteins in the brain. This process, known as protein misfolding and aggregation, leads to the progressive degeneration of nerve cells.
Scientists are now saying that the toxic clumps of protein that trigger Huntington’s Disease spread like a destructive forest fire.
A study, conducted by researchers affiliated with Seattle’s University of Washington School of Medicine, pinpointed the trigger behind this phenomenon. Named the molecular matchstick, the component causes misfolding and aggregation in the brain.
Scientists delved deep into the matter and attained an understanding of nucleation, a critical stage, where misfolded proteins start to aggregate and propagate in neurodegenerative diseases. And during this process, they identified a single molecule that causes a domino effect of destruction.
Huntington’s disease is an inherited disorder that leads to the progressive breakdown of nerve cells in the brain, affecting movement, behavior, and cognitive functions.
The study not only offers hope to those affected by Huntington’s, but also holds promise for advancing research and potential therapies for other related conditions like Parkinson’s and Alzheimer’s.
The individual cell method showed promise
To impede the process, the scientists devised a technique to carry out their experiments within individual cells. They manipulated the genes to generate multiple variations of a protein segment called PolyQ, which was known to turn harmful in Huntington’s disease. These different versions of PolyQ were introduced into the cells, and the researchers closely examined any indications of the proteins folding incorrectly and clumping together.
“It’s sort of like if you’re in a dark room and you’re trying to figure out what the shape of the room is,” Halfmann says. “You just keep bumping into things and eventually you bump into things enough times to figure out exactly what it looks like.”
To counteract the fire, scientists discovered a way that involved flooding cells with specific proteins that prevent more damage from occurring.
“As the disease progresses you’re effectively watching a sort of a forest fire,” Randal Halfmann, an author of the study and a researcher at the Stowers Institute for Medical Research in Kansas City, told NPR. “And you’re trying to figure out what started it.”
“Ultimately, it only matters if we actually create a therapy,” he says. “Otherwise, it’s just academics.”
A new study has found that Huntington’s Disease affects muscles. Photo courtesy of Pexels