Large Aggregates of ALS-causing Protein Might Actually Help Brain Cells

The Dokholyan and Deshmukh labs published a report in PNAS where they examined the neurotoxicity of various SOD1 protein aggregates in a model of Amyotrophic Lateral Sclerosis (ALS). The large SOD1 aggregates, which are a hallmark of ALS, are generally considered to contribute to motor neuron death in ALS. However, the study found that the large SOD1 aggregates are less toxic than the smaller SOD1 trimers. These results suggest that therapies targeted towards the SOD1 trimers, rather than the larger SOD1 fibrils, may be more effective for the treatment of ALS.