Cytosolic aggregates of SOD1 protein are a hallmark of ALS caused by SOD1 mutations, but the role of these aggregates in disease pathogenesis has remained unclear. According to a report in the May 3 Journal of Clinical Investigation by Thomas Brännström and colleagues at Umeå University in Sweden, isolated aggregates of mutant SOD1 injected into the spinal cord of mice expressing human G85R-SOD1 accelerate the onset and progression of disease in these mice. Two different strains of SOD1 aggregates induced the prion-like spreading throughout the spinal cord and brainstem, but the ALS-like symptoms and rate of disease progression differed by strain. These findings, together with similar observations reported using spinal cord homogenates from SOD1 mice (Ayers et al., 2016), suggest that SOD1 aggregates may actively contribute to disease progression and spreading in SOD1 ALS.
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Bidhendi EE, Bergh J, Zetterström P, Andersen PM, Marklund SL, Brännström T. Two superoxide dismutase prion strains transmit amyotrophic lateral sclerosis-like disease. J Clin Invest. 2016 May 3.[Pubmed].