Soak up the SOD. Researchers discovered that cultured neurons build up SOD1 (red) upon taking up misfolded forms of the enzyme. Adapted from Münch et al. (2011).
The buildup of proteins in the brain and spinal cord is a hallmark of ALS. But how these proteins accumulate within the cells of the central nervous system in people with ALS remains a mystery.
Now, researchers from the University of Cambridge in England report that familial ALS type 1-associated superoxide dismutase 1 (SOD1) can spread from neuron to neuron. And once inside these neurons, these misfolded proteins can trigger the aggregation of the cells’ own copies of the enzyme.
These results suggest that ALS may be similar to prion diseases such as the brain disorder Creutzfeldt-Jakob disease. And at the same time, these findings suggest new therapeutic strategies to reduce the buildup of proteins in people with the disease.
Reference
Münch, C., O'Brien, J. and Bertolotti, A. (2011) Prion-like propagation of mutant superoxide dismutase-1 misfolding in neuronal cells. Proceedings of the National Academy of Sciences 108(9), 3548-3553. Abstract | Full Text