Amyotrophic lateral sclerosis (ALS) is a debilitating neurodegenerative disease that affects nerve cells in the brain and spinal cord. Now, researchers have found the clumps of toxic proteins responsible for the onset of ALS. The finding might give a promise to a cure for patients diagnosed with ALS.
The study focused on the genetic mutation of one particular protein called SOD1. It's known that the protein was unique to ALS because it killed neurons within the patients' brains. But how the protein looked remained unclear.
The researchers developed a way to trace brain damage caused by this mutation. Using a combination of algorithms and computer models, they identified SOD1's structure at the damaged sites within live cells. They discovered that SOD1 formed into clumps of three proteins called trimers, which are capable of killing motor neurons. In patients with ALS, it led to gradual paralysis — not only with moving legs and arms, but also with speaking, swallowing, and breathing — and eventual death.
This is a big breakthrough because nobody has known exactly what toxic interactions are behind the death of motor neurons in patients with ALS. It can help to design drugs that would stop the trimers from forming, or sequester them before they can do damage.
Proteins, when clumped into sets of three, are unstable in the body, which hence why they're considered a mutation. The researchers considers it's the reason behind SOD1's toxicity in the brain. The proteins interact with other neurons and wind up damaging them permanently, causing ALS's characteristic degeneration.
It's difficult to treat neurodegenerative diseases. Unlike many cancers and other conditions, we currently have no leverage against these neurodegenerative diseases. The new study may not only benefit those affected by ALS, but also shed light on other neurodegenerative diseases. Neurodegenerative diseases have something in common. The results of the study seem to corroborate what is known about Alzheimer's. Moreover, further study could help to understand the causes of other neurodegenerative diseases.
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