University of North Carolina School of Medicine researchers studying proteins that mutate and kill neurons in the brain and spinal cord have figured out a way to stabilize the proteins by in essence reversing the mutation.
In the medical journal Structure, UNC School of Medicine researchers said they stabilized those proteins, called SOD1, by mutating them again. Those changes stopped the proteins from forming clumps, which is when they damaged and killed neurons.
Properly functioning SOD1 proteins work in two pairs rather than in clumps of three.
Researchers were able to create a mutation that simulated the addition of a phosphate group to stabilize the proteins. The newly mutated proteins did not harm neurons.
The discovery could be helpful to patients with amyotrophic lateral sclerosis (ALS) related to SOD1 protein mutations, but researchers said learning how to stabilize proteins such as SOD1 could also benefit patients with other forms of ALS.
