Scientists at Umeå University in Sweden have found that superoxide dysmutase (SOD1), a protein that may cause amylotophic lateral sclerosis (ALS) spreads and clumps when injected into mice. The protein deposits also cause ALS-like symptoms. The report, titled “Two superoxide dismutase prion strains transmit amyotrophic lateral sclerosis-like disease” appeared May 3, 2016 in the Journal of Clinical Investigation.
“The occurrence of SOD1 aggregates in nerve cells in ALS patients has been known for a while,” remarked Thomas Brännström, professor of Pathology at Umeå University and the article’s principal author.
“But it has long been unclear what role the SOD1 aggregates play in the disease progression in humans carrying hereditary traits for ALS. We have now been able to show that the SOD1 aggregates start a domino effect that rapidly spreads the disease up through the spinal cord of mice. We suspect that this could be the case for humans as well” he added.